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NEUROLOGY Basics
NEUROLOGY Basics
Medulla Oblongata
Relays sensory information to thalamus and to other portions of
the brain stem
Autonomic centres for regulation of visceral function
(cardiovascular, respiratory and digestive system activities).
Cerebellum
Coordinates complex somatic motor patterns
Adjusts output of other somatic motor centres in brain and
spinal cord.
Obeys commands
Localising to pain
Apply a painful stimulus to the supraorbital nerve, e.g. rub
thumb nail in the supraorbital groove, increasing pressure until a
response is obtained. If the patient responds by bringing the
hand up beyond the chin = ‘localising to pain’. (Pressure to nail
beds or sternum at this stage may not differentiate ‘localising’
from ‘flexing’.)
Flexing to pain
Extending to pain
If in response to the same stimulus elbow extension occurs,
record as ‘extending to pain’. This is always accompanied by
spastic flexion of the wrist. None. Before recording a patient at
this level, ensure that the painful stimulus is adequate. During
examination the motor response may vary. Supraorbital pain
may produce an extension response, whereas fingernail pressure
produces flexion. Alternatively one arm may localise to pain; the
other may flex. When this occurs record the best response during
the period of examination (this correlates best with final
outcome). For the purpose of conscious level assessment uses
only the arm response. Leg response to pain gives less consistent
results, often producing movements arising from spinal rather
than cerebral origin.
MEMORY TEST
6
Testing requires alertness and is not possible in a confused or
dysphasic patient.
IMMEDIATE memory – Digit span – ask patient to repeat a
sequence of 5, 6, or 7 random numbers.
RECENT memory – Ask patient to describe present illness,
duration of hospital stay or recent events in the news.
REMOTE memory – Ask about events and circumstances
occurring more than 5 years previously.
VERBAL memory – Ask patient to remember a sentence or a
short story and test after 15 minutes.
VISUAL memory – Ask patient to remember objects on a tray and
test after 15 minutes.
Note: Retrograde amnesia – loss of memory of events leading up
to a brain injury or insult.
Post-traumatic amnesia – permanent loss of memory of events for
a period following a brain injury.
7
by testing reading acuity through a pinhole. This concentrates a
thin beam of vision on the macula.
Visual fields
Gross testing by CONFRONTATION. Compare the patient’s fields
of vision by advancing a moving finger or, more accurately, a red
5 mm pin from the extreme periphery towards the fixation point.
These maps out ‘cone’ vision. A 2 mm pin will define central field
defects which may only manifest as a loss of colour perception.
In the temporal portion of the visual field the physiological blind
spot may be detected. A 2 mm object should disappear here.
The patient must fixate on the examiner’s pupil. OCULOMOTOR
(III), TROCHLEAR (IV) AND ABDUCENS (VI) NERVES A lesion of
the III nerve produces impairment of eye and lid movement as
well as disturbance of pupillary response. Pupil: The pupil dilates
and becomes ‘fixed’ to light. Shine torch in affected eye –
contralateral pupil constricts (its III nerve intact). Absent or
impaired response in illuminated eye.
9
Ask patient to rotate head against resistance. Compare power
and muscle bulk on each side. Also compare each side with the
patient pulling head forward against resistance. N.B. The left
sternomastoid turns the head to the right and vice versa.
Trapezius
Ask patient to ‘shrug’ shoulders and to hold them in this position
against resistance. Compare power on each side. Patient should
manage to resist any effort to depress
Power
Muscle weakness. The degree of weakness is ‘scored’ using the
MRC (Medical Research Council) scale.
10
Score 0 – No contraction
Score 1 – Flicker
Score 2 – Active movement/gravity eliminated
Score 3 – Active movement against gravity
Score 4 – Active movement against gravity and resistance
Score 5 – Normal power
SENSATION
Pain
Pin prick with a sterile pin provides a simple method of testing
this important modality. Firstly, check that the patient detects
the pin as ‘sharp’, i.e. painful, then rapidly test each dermatome
in turn. Memorising the dermatome distribution is simplified by
noting that ‘C7’ extends down the middle finger.
If pin prick is impaired, then more carefully map out the extent of
the abnormality, moving from the abnormal to the normal areas.
Light touch
This is tested in a similar manner, using a wisp of cotton wool.
Temperature
Temperature testing
Joint position sense
Hold the sides of the patient’s finger or thumb and demonstrate
‘up and down’ movements. Repeat with the patient’s eyes closed.
Ask patient to specify the direction of movement. Ask the patient,
with eyes closed, to touch his nose with his forefinger or to bring
forefingers together
Vibration
Place a vibrating tuning fork (usually 128 c/s) on a bony
prominence, e.g. radius. Ask the patient to indicate when the
vibration, if felt, ceases. If impaired, move more proximally and
repeat. Vibration testing is of value in the early detection of
demyelinating disease and peripheral neuropathy, but otherwise
is of limited benefit. If the above sensory functions are normal
and a cortical lesion is suspected, it is useful to test for the
following:
Two point discrimination: the ability to discrimination.
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Two point discrimination: the ability to discriminate two
blunt points when simultaneously applied to the finger, 5
mm apart (cf, 4 cm in the legs).
Sensory inattention (perceptual rivalry): the ability to detect
stimuli (pin prick or touch) in both limbs, when applied to
both limbs simultaneously.
Stereognosis: the ability to recognise objects placed in the
hand.
Graphaesthesia: the ability to recognise numbers or letters
traced out on the patient.
REFLEXES
Biceps jerk C5, C6 roots. Musculocutaneous nerve
CO-ORDINATION
Inco-ordination (ataxia) is often a prominent feature of cerebellar
disease prior to testing; ensure that power and proprioception are
normal.
CO-ORDINATION
Ask patient to repeatedly run the heel from the opposite knee
down the shin to the big toe. Look for ATAXIA (in co-ordination).
Ask patient to repeatedly tap the floor with the foot. Note: any
DYSDIADOCHOKINESIA (difficulty with rapidly alternating.
GAIT
Note:
Length of step and width of base Normal
Abnormal leg movements (e.g. excessively high step)
Instability (gait ataxia)
Associated postural movements (e.g. pelvic swinging.
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13
CHAPTER 3
LESIONS OF PYRAMIDAL SYSTEM.
NEUROANATOMY, PYRAMIDAL TRACT LESIONS
Introduction
The central nervous system (CNS) is a complex network of
components that allow an organism to interact with its
environment. It is made up of multiple different parts, each of
which plays a different role. Primarily, the CNS is formed by the
upper motor neurons (UMN) which carry signals for movement
down to the lower motor neurons (LMN) which signal the muscles
to either contract or relax. The UMN further subdivides into
multiple tracts, each of which has specific functions within the
body. Specifically, the pyramidal tract is the main pathway that
carries signals for voluntary movement. Lesions to the pyramidal
tract can lead to devastating consequences such as spasticity,
hyperactive reflexes, weakness, and a Babinski sign (stroking the
sole of the foot causes the big toe to move upward). These
symptoms are all characteristic of an upper motor neuron lesion.
However, certain symptoms are specific to a pyramidal tract
lesion.
Surgical Considerations
Pyramidal tract lesions can have devastating consequences if not
discovered quickly. The most important aspect of surgery focuses
on determining the location and cause of the lesion, which will
help dictate the procedure. A detailed history and physical exam
will aid in guiding this process. Pyramidal tract lesions will
present very similarly to upper motor lesions with symptoms
such as hyperreflexia, weakness, spasticity, and a Babinski sign.
Damage to the corticobulbar tract can present with additional
symptoms of lower facial weakness and changes to speech. Initial
treatment for these lesions is typically intensive rehabilitation
and exercise. They can also be managed with medical
interventions such as botulinum toxin, benzodiazepines, and
baclofen, which can all help to decrease the spasticity and
contractures to improve functionality and quality of life in
patients. It is only when these measures fail, and in the cases of
a severe and life-threatening emergency, that surgery becomes a
consideration.
Clinical Significance
Pyramidal tract lesions can occur from any type of damage to the
brain or spinal cord. They can result from a variety of injuries
and diseases such as strokes, abscesses, tumors, hemorrhage,
meningitis, multiple sclerosis, or trauma. Damage to the
corticospinal tract will present similarly to upper motor lesion
syndrome and will present with symptoms such as spasticity,
clonus, hyperreflexia, and Babinski sign. Damage to the
corticobulbar tract can present with pseudobulbar palsy or
damage to cranial nerves VII or X.
Pseudo-bulbar Palsy
The corticobulbar tract bilaterally innervates most of the cranial
nerves, except VII and XII, which means that for symptoms to
arise from damage to these nerves, both sides of the
corticobulbar tract must be injured as is the case in pseudo-
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bulbar palsy. Symptoms in this condition may include slow
speech, dysphagia (difficulty swallowing), dysarthria (difficulty
speaking), spastic tongue, and pseudobulbar affect
(uncontrollable episodes of laughing or crying).
Other Issues
There are a wide variety of pathologies associated with pyramidal
tract lesions. They can be the result of many diseases including
stroke, amyotrophic lateral sclerosis, multiple sclerosis, and
central pontine myelinolysis.
Stroke
Cerebrovascular accidents, or strokes, are caused by occlusion of
blood flow to a particular area of the brain. They divide into
either an ischemic stroke or hemorrhagic stroke. Ischemic
strokes are the sudden interruption of blood supply to a
structure due to occlusion or obstruction by a thrombus or
embolus. Hemorrhagic strokes result from the rupture of a blood
vessel leading to bleeding into the brain. Because the pyramidal
tract is such a large structure and receives blood supply from so
many different arteries, any occlusion to these supporting
arteries can lead to a wide variety of symptoms.
Multiple Sclerosis
Multiple sclerosis is a demyelinating autoimmune disease of the
nervous system. Its manifestations show a wide range of
symptoms such as double vision, muscle weakness, coordination
trouble, or cognitive disturbance. It is the most common CNS
autoimmune disorder, and there currently is no cure.
Management centers on improving function after an attack, and
preventing recurrent attacks.
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CHAPTER 4
CEREBROVASCULAR ACCIDENT CVA
CAUSATION
Strokes are usually classified by the mechanism and location of
the vascular damage. The two broad causes are ischemia and
hemorrhage. Ischemic strokes result from a blockage of a
cerebral vessel and can further be categorized as caused by
thrombosis or embolism. Thrombosis is the stenosis or occlusion
of a vessel, usually as a result of atherosclerosis. This occlusion
is typically a gradual process, often with preceding warning
signs, such as transient ischemic attack (TIA). An embolism is
dislodged platelets, cholesterol, or other material that forms at
another location, travels in the bloodstream, and blocks a
cerebral vessel. Ischemic strokes are the most common type,
accounting for about 87% of strokes (American Stroke
Association, 2011). Hemorrhagic strokes result from a rupture of
a weakened cerebral blood vessel. In such strokes, blood
accumulates outside of the vascular space and compresses
surrounding brain tissue. Hemorrhagic strokes are either
intracerebral (bleeding into the brain itself) or subarachnoid
(bleeding into an area surrounding the brain). Aneurysms and
arteriovenous malformations are the most common types of
weakened blood vessels causing hemorrhagic strokes (American
Stroke Association, 2011). Hemorrhagic strokes are less common
(an estimated 13% of strokes), but they result in a higher
mortality rate than ischemic strokes (Roger et al., 2011).
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Location of Involvement
Most lesions are either anterior circulation strokes, which
present signs and symptoms of hemispheric dysfunction, or
posterior circulation strokes, which display signs and symptoms
of brainstem involvement (Simon, Greenberg, & Aminoff, 2009).
Another distinction related to location of CVA is whether the
lesion results from large-vessel or small-vessel disease.
Thrombosis occurs most often in the large cerebral blood vessels.
Small-vessel, or lacunar, strokes are very small infarctions that
occur only where small arterioles branch off the larger vessels
(National Institutes of Health, 2011).
INCIDENCE
Stroke is the third leading cause of death in the United States
and a leading cause of chronic disability among adults. Of the
estimated 795,000 persons who have first or recurrent CVAs in
the United States each year, approximately two-thirds survive,
bringing the number of stroke survivors in the U.S. population at
any one time to over nine million (American Stroke Association,
2011). Stroke is the most common neurological diagnostic
category among patients seen by occupational therapists
(National Board for Certification in Occupational Therapy, 2008).
The projected aging of the U.S. population is expected to raise the
incidence of stroke, because about two-thirds of all strokes occur
in those older than age 65 years (National Institutes of Health,
2011).
MEDICAL MANAGEMENT
Acute stroke care focuses on determining the cause and site of
the stroke, preventing progression of the lesion, reducing cerebral
edema, preventing secondary medical complications, and treating
acute neurological symptoms (Bartels, 2011). Techniques of
diagnostic imaging, including computed tomography (CT) and
magnetic resonance imaging (MRI), can distinguish ischemic from
hemorrhagic lesions and define their location, size, and vascular
territory (National Institutes of Health, 2011). In acute ischemic
stroke, treatment concerns include restoration of blood flow and
limitation of neuronal damage. Medications, including
antithrombotics and thrombolytics, are the most common
medical intervention for stroke (National Institutes of Health,
2011). Antithrombotics include antiplatelet drugs (such as
20
aspirin) and anticoagulation drugs (such as heparin) and are
used to prevent further clotting or thrombosis. After stroke,
physicians mainly prescribe antithrombotics for prevention,
because first time stroke survivors are at a high risk for recurrent
strokes (National Institutes of Health, 2011). Thrombolytic drugs,
such as tissue plasmino-gen activator (tPA), help reestablish
blood flow to the brain by dissolving clots in cerebral vessels. A 5-
year trial conducted by the National Institute of Neurological
Disorders and Stroke found that appropriately selected subjects
treated with TPA within 3 hours of onset of stroke symptoms
were at least 33% more likely than patients given a placebo to
recover from their stroke with little or no disability (National
Stroke Association, 2011). The use of thrombolytics is limited,
however, by the associated increased risk of hemorrhage and the
fact that they must be administered within a 3-hour window after
stroke onset for effectiveness. It is estimated that only 3%–5% of
per-sons suffering a stroke will seek medical attention in time for
possible TPA treatment (American Stroke Association, 2011).
With hemorrhagic stroke, acute treatment includes control of
intracranial pressure, prevention of rebleeding, maintenance of
cerebral perfusion, and control of vasospasm. Surgery or
endovascular procedures are often recommended to limit damage
(National Stroke Association, 2011).
ASSESSMENT
Numerous evaluations exist to identify stroke impairments and
disability. Assessment practices vary greatly among therapists
caring for individuals post stroke, and no single test is
universally accepted (Gresham et al., 1995). To help in the
selection and ordering of assessment tools, therapists are guided
by models of practice and evidence-based practice guidelines. The
OT assessment of a patient post stroke includes both
determination of the occupational profile (the roles, tasks, and
activities important to that individual) and analysis of the
individual’s occupational performance (his or her competence in
22
performing these valued roles, tasks, and activities). Therapists
consider each individual and the individual’s stage of recovery
when determining whether to follow a bottom-up or a top-down
approach to client evaluation. During the acute stage,
assessments of client factors and performance skills are most
critical in deter-mining immediate post hospital placement.
During the rehabilitation and community-reentry stages,
therapists must also consider activity demands, contexts,
patterns of performance, and integration of skills in order to help
clients reach goals for quality of life and participation in
meaningful roles (Sabari & Lieberman, 2008). Therapists can use
available practice guidelines to assist in the selection of
assessment tools. Recommended guide-lines are the American
Occupational Therapy Association’s Occupational Therapy
Practice Guidelines for Adults with Stroke (Sabari & Lieberman,
2008) and Appendix D of the American Heart
Association/American Stroke Association’s (AHA/ASA)
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CHAPTER 5
INTRODUCTION AND APPLICATION OF APPROACHES/FORS
USED IN NEUROLOGICAL REHABILITATION SENSORIMOTOR
TREATMENT APPROACHES
INTRODUCTION
Sensory integration (SI) theory is a dynamic and ecological theory
that specifies the critical influence of sensory processing on
human development and function. This theory emphasizes a
person’s ability to appropriately process sensory information from
the body and integrate it with information about what is going on
around the person so that he or she can effectively acton the
environment. It contributes to our understanding of how
sensation affects learning, social-emotional development, and
neurophysiological processes such as motor performance,
attention, and arousal. Occupational therapists commonly use
sensory integration theory in practice as a frame of reference that
can be used to both assess and intervene with people who have
sensory integrative or sensory processing dysfunction that
adversely affects function (Parham & Mailloux, 2005). It is
important to be clear what one is referring to when using the
term sensory integration, as this singular term has been used to
refer to a theory, a neurological process, a disorder, and an
intervention approach (Bundy, 2002; Mulligan, 2003). The
theoretical principles that support the use of this frame of
reference draw on evidence from neuroscience as Dr. Ayres
recognized that behavior and emotions are regulated by brain
mechanisms. Advances in neuroscience continue to expand and
support Dr. Ayres’ original hypotheses, contributing to evidence-
based practice (Bauman, 2005; Schneider, 2005). Dynamic
systems theories have emerged that provide an under- standing
of the complexity of development (Thelen & Smith, 1994) and
help to explain the complexity of the way in which this
intervention effects change in children (Spitzer, 1999). SI is now
commonly applied in educational settings with children who have
learning and behavior disorders (American Occupational Therapy
Association, 2003), as well as in private settings. While education
in sensory integration theory and intervention principles is part
of the entry-level curricula for occupational therapists and
occupational therapy assistants, postgraduate training is
recommended for those specializing in the use of SI intervention
25
in clinical practice. Occupational therapists with sensory
integration certification and mentorship with a master clinician
are best prepared to evaluate and provide SI intervention.
Occupational therapy assistants may provide intervention using
SI principles with appropriate supervision by an occupational
therapist.
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CHAPTER 6
SENSORY INTEGRATION THEORY
Neuroplasticity
Dr. Ayres’ appreciation of the importance of experience as a
major determinant of cortical organization and resulting function
remains a bedrock principle in the field of neuroscience today.
This “experience-dependent plasticity in the cerebral cortex
reflects the importance of learning in our mental life and
behaviors” (E. K. Miller, 2000, p. 1067). During the period when
Dr. Ayres studied neuroplasticity, researchers thought that the
brain’s ability to be modified was robust during childhood but,
after a critical period of development, became quite limited. It is
now known that the brain has the capacity to be modified
throughout the life course (Bear, Connors, & Paradiso, 2006;
Gilbert & Wiesel, 1992).
Developmental Progression
Viewing development as a process that the brain undergoes, Dr.
Ayres noted that “each child’s brain is designed to follow an
orderly, predictable, interrelated sequence of development that
results in the capacity for learning” Given an enriched,
supportive environment, children will grow and develop sensory
and motor memories that help the children adapt to their own
30
growth and interests in the context of an ever-changing
environment. Whereas Dr. Ayres focused on the first seven years
of life as the time frame in which this occurred, we now know
that the brain continues to develop throughout the life course
(Bear et al., 2006; Gilbert & Wiesel, 1992). A critical aspect of
this process is that the child experience sensations, which places
a demand on the brain to organize the incoming stimuli into
percepts. Depending on the context in which the stimuli are
experienced, the child might focus on and attend to the input
(sensory detection as a central nervous system process; sensory
registration as inferred from the child’s behavior), or if they are
not relevant, the stimuli might be ignored (inhibition centrally,
sensory modulation behaviorally) (Lane, Miller, & Hanft, 2000;
Miller & Lane, 2000). For example, a child who is riding a bicycle
typically attends to visual and auditory inputs along the ride
(sensory registration) while ignoring the feel of the shirt as it is
blown by the wind (inhibition). If the child’s brain is unable to
organize incoming sensation efficiently, these “filtering out” and
“attending to” processes might be inadequate. In this example,
the child who is unable to tolerate the tactile input from the
shirt’s movement against the body will have difficulty attending
to the important visual and auditory information in the
environment that are necessary for both safety and skill. Until
the tactile sensation is discriminated as “safe,” the child is
considered to remain in a more primitive “fight- or-flight” mode.
In general, this primitive state under- mines both the
development of skills and emotions as it is through the ability of
the brain to organize sensations that “the child gains control over
his emotions” (Ayres,
Adaptive Response
Our ability to make adaptive responses to ever-changing
environmental demands and challenges allows us to learn
something new and, in turn, change the environment (Ayres,
1972, 1979, 2005). Spitzer (1999) discusses the congruence
between the concepts of self-organization in dynamic systems
theory with Dr. Ayres’ concept of the adaptive response in
sensory integration theory. In both theories, feedback from the
individual’s spontaneous, active adjustments contributes to self-
organization of the brain (Smith & Thelen, 1993; Ayres, 1979,
2005). Dr. Ayres stressed the importance of “organizing adaptive
31
responses to increasing complexity” as a key component of
intervention. She was guided by neuroscience research
suggesting that inefficiency in synaptic activity along anatomical
pathways might be contributing to poor integration of sensory
information and proposed that focusing on eliciting a response
that was not yet well developed might enhance synaptic function
(Ayres, 1972; Katz & Shatz, 1996; Schlaug, 2001). While an
adaptive response occurs most typically during a motor task, it is
also apparent with demands arising in other domains (e.g.,
emotional regulation, cognitive, and social interaction). “Therapy
using a sensory integration approach is a natural process”
Postural Adaptation
Postural adaptation, or postural control, refers to the individual’s
ongoing ability to achieve, maintain, or restore an upright
position against gravity (balance) for stability during activities or
changes in body position (Pollock et al., 2000). The recognition
and treatment of deficits in postural adaptation constitute an
important aspect of therapy for stroke patients because so many
daily living tasks (e.g., putting on socks, getting in and out of a
bathtub, housework, and participating in sports) depend on this
skill. Evaluation and treatment limited to a patient securely
supported in bed or in a wheelchair fail to address most usual
daily tasks that require dealing with gravity. A person with
hemiplegia typically has decreased motor control, poor bilateral
32
and sensory integration, and impaired automatic postural
responses (Oliveira et al., 2008). As a result, the patient must
devote increased effort to remaining upright, with decreased
ability to focus on purposeful tasks. When engaging in a
challenging activity, the hemiplegic patient often resorts to
compensatory strategies to help maintain stability, such as using
upper extremities for support (Oliveira et al., 2008). Postural
adaptation factors and skills can best be observed during the
performance of meaningful functional activities, although the
Berg Balance Scale.
Somatosensory Assessment
During evaluation of sensory deficits in the person with stroke, it
is important to remember that sensation is a component of
function that is only a focus for treatment when it relates to the
ability to perform usual daily living tasks. When somatosensory
disturbances are present, they usually accompany motor
impairment in the same anatomic distribution (Stein &
Brandstater, 2010). Most tests of sensation require attention,
recognition, and response to multiple stimuli; therefore, sensory
testing is difficult in patients with aphasia, confusion, and other
cognitive deficits. It is often necessary to determine the patient’s
level of comprehension and communication, including yes/no
reliability. An expressively aphasic patient can nod, gesture,
point to written or pictured cues, or select a stimulus object from
an array of objects. When testing with standard procedures is not
possible, information may still be gained from observing a
patient’s reactions to the testing. The presence of gross protective
33
sensation (flinching when pricked with a sharp pin) can be
documented even if discriminatory perception cannot be
determined. Patients who have had mild CVAs and who have
intact primary sensory awareness may need to be tested for more
subtle discriminatory problems using two-point discrimination
testing or the Moberg Pick-up Test (Dellon, 1981). Such testing is
indicated when motor return is good, but hand dexterity remains
impaired. Chapter 9 provides de-tails of sensory assessment.
Voluntary Movement
Determining the amount and quality of voluntary movement a
patient can produce is one of the first steps in assessing
movement potential (Warren, 1991). The patterns of motion
available are different for each stroke patient. Movement can
change dramatically or subtly with time; hence, it requires
careful reassessment throughout recovery. Factors to consider
when evaluating motor control of the involved upper extremity
include the following:
Can the patient perform reflexive but not voluntary
movement? Example: Patient demonstrates active el-bow
extension in the involved arm when balance is disturbed
(equilibrium reaction) or flexes the hemi paretic elbow while
yawning (associated reaction) but can perform these
movement on request Do proximal segments (neck, trunk,
shoulder, hip) stabilize as needed to provide firm support for
movement of the distal parts, or do they substitute for distal
movement? Example: A patient can raise his hemi paretic
arm only with pronounced lateral bending of the trunk and
excessive elevation of the shoulder girdle.
Can voluntary movement be performed unassisted against
gravity, or is it possible only with assistance in the form of
positioning, support, or facilitation? Example: A patient can
bring her hand to her mouth only by flexing her elbow in a
horizontal plane with gravity eliminated.
35
Can voluntary movement be performed in an isolated
fashion or only in a synergistic pattern? Example: A patient
can reach for an object on a table only with a pat-tern of
shoulder abduction, elbow flexion, and trunk flexion rather
than with the more efficient pattern of shoulder flexion and
elbow extension.
Can reciprocal movement (the ability to perform
agonist/antagonist motion in succession in an individual
joint) be performed with practical speed and precision?
Examples: A patient cannot produce a smooth pattern of
elbow extension-flexion-extension needed to grasp a glass,
take a drink, and set it back on the table but can perform
each movement separately. A patient cannot perform the
rapid alternating movements necessary to brush teeth.
One of the major movement difficulties following stroke is
attaining the capacity and ability to isolate and control single
muscle actions and combine them in a pat-tern appropriate for
the task at hand. In motor patterns typical in hemiplegia,
movement initiated in one joint results in automatic contraction
of other muscles linked in synergy with that movement. This
results in limited, stereotyped movement patterns rather than
adaptive, selective motions. In Brunnstrom’s theory of hemiplegic
limb synergies (Brunnstrom, 1970), typical stereotyped patterns
are described as flexor or extensor synergy patterns according to
the motion at the elbow. The flexor synergy presents with
scapular retraction and/or elevation, shoulder abduction and
external rotation, elbow flexion, and forearm pronation. The
extensor synergy presents with scapular protraction, shoulder
horizontal adduction and internal rotation, elbow extension, and
forearm pronation. Wrist and hand position varies (Brunnstrom,
1970). There is considerable variation in synergistic patterning,
and other causes of abnormal stereotyped patterns include
compensatory movements, unnecessary movement, muscle
tension resulting from exertion or stress, and movement in
response to gravity (e.g., pronation). According to Brunnstrom
(1970), movement recovery after stroke is determined by an
individual’s ability to move independently of synergies. More
contemporary clinical studies suggest that in addition to
pathological limb synergies, loss of strength or centrally mediated
impaired interjoint coordination can contribute to movement
disturbances and impaired function (Welmer, Holmqvist, &
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Sommerfeld, 2006). A study designed to look at the extent
abnormal limb synergies influence voluntary movements of hemi
paretic patients with first-time stroke found that 3 months after
stroke, 13% of all subjects moved within synergies (Welmer,
Holmqvist, & Sommerfeld, 2006). Several methods for evaluating
voluntary movement post stroke are described in Chapter 8 and
online Chapters A, B, and C. Valid and reliable tools for
evaluating voluntary movement post stroke recommended by
both the AHA/ASA clinical guideline and the AOTA practice
guideline are the Fugl-Meyer Assessment of Motor Function
(Fugl-Meyer et al., 1975) and the Motor Assessment Scale (Carr
et al.,1985). The Fugl-Meyer Assessment is an adaptation of
Brunnstrom’s original Hemiplegia Classification and Progress
Record (Brunnstrom, 1970) and incorporates Brunnstrom’s six
stages of motor recovery with an underlying predicted sequence
of recovery. The Fugl-Meyer Assessment of the Upper Extremity
(FMA-UE) is the most commonly used research assessment to
describe upper extremity motor impairment and evaluate the
success of new interventions, but its focus on synergy patterns
no longer forms the basis for newer function-oriented treatments
(Woodbury et al., 2007). After investigating the dimensionality
and construct validity of the FMA-UE, researchers suggest that
assessment of reflexes in the FMA-UE gives little information
about volitional movement. They also challenge the stepwise
orderly sequence of motor recovery described by Brunnstrom and
Fugl-Meyer and suggest instead that “UE motor behavior during
recovery may be a dynamic interaction of neural factors with the
task-specific difficulty of a movement” (Woodbury et al., 2007, p.
720).
TREATMENT
A careful interpretation of evaluation results helps determine a
patient’s assets and deficits in areas of occupational functioning.
General goals of OT intervention with stroke survivors are to
prevent secondary impairments, restore performance skills,
modify activity demands and contexts as necessary, promote a
healthy and satisfying lifestyle, and maintain available
performance and health (Sabari & Lieberman, 2008). Intervention
is always a collaborative process between therapist and
client/family or caregiver. Possible goals for individuals
recovering from stroke include the following:
The patient will gain competence in valued and necessary
BADL and IADL in order to perform at the highest level of
independence possible in the desired post discharge setting.
The patient will improve postural control in order to perform
daily living tasks requiring balance and changes in body
position.
The patient will gain increased somatosensory perception
and/or will employ compensatory strategies in order to
perform ADL safely.
The patient and/or caregiver will demonstrate appropriate
management techniques for the hemiparetic upper
extremity to prevent pain and other secondary mechanical
or physiological movement restrictions.
The patient will gain the necessary strength, endurance,
and control of movement of the involved upper extremity in
order to use the involved upper extremity spontaneously
during the performance of ADL.
The patient will gain visual function or will employ
compensatory strategies in order to safely resume previously
performed ADL.
The patient will improve motor planning ability in order to
relearn old methods or learn new methods of performing
ADL.
38
The patient and/or caregiver will demonstrate appropriate
strategies for improving or compensating for cognitive
deficits during the performance of ADL.
The patient and/or caregiver will be able to verbalize the
reality and impact of emotional reactions to stroke and
identify coping strategies or resources to help ad-just to
living with a stroke.
The caregiver will demonstrate appropriate methods and
problem-solving strategies for assisting the patient with ADL
and with home activities to improve/pre-serve performance
skills.
The patient will gain competence in tasks and activities
necessary to resume valued roles or to assume new
meaningful roles in the community.
Intervention will vary with the patient’s stage of recovery,
intervention setting, living environment, extent of impairment,
and personal goals and preferences (Sabari & Lieberman, 2008).
Acute Phase
Stroke rehabilitation begins “as soon as the diagnosis of stroke is
established and life-threatening problems are under control”
(Duncan et al., 2005, p. e104). Length of stay in acute hospital
beds is typically just long enough for necessary diagnostic tests,
for initiation of appropriate some control over the environment
and begin to improve occupational functioning and component
abilities and capacities (Gresham et al., 1995). Even at this early
stage, the occupational therapist’s assessment of a patient can
help determine the most appropriate setting for re-habilitation
and discharge. The goals of discharge planning during the acute
phase are to determine the need for postacute rehabilitation,
arrange the best possible living environment, and ensure
continuity of care after discharge (Gresham et al., 1995).
Skin Care
It is estimated that up to 21% of patients with stroke develop
pressure sores (Langhorne et al., 2000). Those who are comatose,
39
malnourished, or incontinent or who have diabetes, peripheral
vascular disease, abnormal sensation, severe paralysis, or muscle
spasticity are at greatest risk (Duncan et al., 2005). The
occupational therapist helps patients maintain skin integrity by
doing the following:
Using proper transfer and mobility techniques to avoid
undue skin friction
Recommending appropriate bed and seated positioning and
participating in scheduled position changes as needed
Assisting with wheelchair and seating selection and
adaptation
Teaching patient and caregiver precautions to avoid injury
to insensitive skin and involved side of body
Watching for signs of skin pressure or breakdown on a
patient (bruising, redness, blisters, abrasions, ulceration),
especially over bony areas, and alerting nursing or medical
staff as appropriate
Rehabilitation Phase
Part of discharge planning during the acute phase of stroke is
screening for rehabilitation services. The AHA/ASA guideline
recommends that “patients who have sustained an acute stroke
should receive rehabilitation services if their poststroke
functional status is below their prestroke status, and if there is a
potential for improvement” (Duncan et al., 2005, p. e118).
Rehabilitation choices depend on a patient’s condition, the social
support system, and the re-sources available in a community. To
qualify for further treatment in an inpatient rehabilitation facility,
a patient must require active and ongoing intervention of multiple
therapy disciplines, require an intensive rehabilitation therapy
program (generally consisting of at least 3 hours of therapy per
day at least 5 days per week), and reasonably be expected to
actively participate in and significantly benefit from the
rehabilitation program (U.S. Department of Health and Human
Services, 2011). Patients who do not qualify for this level of
rehabilitation may receive multidisciplinary rehabilitation
services at a skilled nursing facility or long-term care facility or
treatment by one or more disciplines in home care or in an
outpatient clinic. During this phase of recovery, the patient and
family are focused on getting better and are usually more
concerned with recovering lost function than on adapting to a life
of chronic disability (Sabari, 1998). Successful OT intervention
coordinates a patient’s striving for restoration of function with
the potential for compensation and alternative occupational roles.
43
Cognitive skills and emotional reactions (sequencing,
attention span, frustration tolerance, motivation) ADL
training with stroke patients begins with simple tasks and
gradually increases in difficulty as a patient gains
competency (Gresham et al., 1995). Several studies
discerned a hierarchy of achievement of self-care skills.
Results of one study showed that bathing, dressing, and
climbing stairs were the activities for which stroke survivors
most often required assistance, with 32% of patients
needing help with bathing, 25.5% needing help with
dressing, and 32% requiring assistance with stairs
12 months poststroke (Carod-Artal et al., 2002). Aspects of
dressing that are particularly difficult for stroke patients are
putting a sock and shoe on the affected foot, lacing shoes,
and pulling up trousers or pants (Walker & Lincoln, 1990).
A study that investigated the relationship between dressing
abilities and cognitive, perceptual, and physical deficits
found that, in general, lower extremity dressing correlates
more with motor performance, and upper extremity dressing
correlates more with cognitive or perceptual performance
(Walker & Lincoln, 1991). Adaptive devices should be
considered if they increase simplicity, independence, and
safety for the patient or caregiver.
As the patient progresses, occupational performance tasks other
than basic self-care should be addressed, particularly if the
patient expects to return to independent community living
(Duncan et al., 2005). IADL tasks such as homemaking, home
management, and community. Mobility involves greater
interaction with the physical and social environment and require
higher level problem-solving and social skills than BADL tasks
(Carod-Artal et al., 2002). Chapter 25 discusses specific
techniques for regaining independence in BADL/IADL for those
with loss of the use of one side of the body. Vocational interests,
including adapted methods of continuing familiar hobbies, are an
important area of treatment. Many stroke survivors are faced
with increases in leisure time because of the inability to go back
to work; however, a reduction in social and leisure participation
commonly occurs after stroke (Lai et al., 2002).
44
Performance-component goals are based on the impairments
associated with an individual’s stroke and are directly linked to
occupational performance goals. The goals and modalities used to
address these component deficits must be purposeful and
meaningful from the patient’s point of view (Trombly,
1995/2011). Therapists use occupation-based interventions,
purposeful activity, and preparatory methods to help patients
achieve long-term occupational goals (AOTA, 2008). Thus, in
addition to direct practice of dressing and grooming activities, a
patient may be engaged in a floor game to improve sitting balance
needed to don socks, or use therapy putty for resistive grasp
activities to strengthen muscles needed to squeeze a tube of
toothpaste. Treatments for stroke deficits are described
individually in the following sections, but most patients in
rehabilitation programs have multiple interacting problems
requiring efficient, integrated intervention plans that
simultaneously address several deficit areas.
Postural Adaptation
The ability to make automatic postural adjustments, including
trunk control and the maintenance of balance, is a prerequisite
for successful performance of occupational tasks. A systematic
review of recent evidence related to the effect of balance training
on balance performance among individuals poststroke supports
the use of balance training for individuals with moderately severe
stroke (Lubetzky-Vilnai & Kartin, 2010). Part of the occupational
therapist’s role in training a patient with stroke in ADL
independence poststroke is in understanding each patient’s
particular strengths and weaknesses regarding stability and
mobility. For ex-ample, some clients may be challenged in
maintaining sitting balance on the side of a bed for the duration
of eating an entire meal, whereas others may be ready to im-
prove dynamic standing balance in order to cast a fishing line or
play golf. Therapists should teach patients the safest, most
effective and efficient “ready” position for engaging in activities.
45
CHAPTER 7
BRUNNSTROM APPROACH
BRUNNSTROM’S MOVEMENT THERAPY
48
CHAPTER 8
ROOD AND PROPRIOCEPTIVE NEUROMUSCULAR
FACILITATION APPROACHES
51
CHAPTER 9
NEURODEVELOPMENTAL THERAPY (THE BOBATH
APPROACH)
57
CHAPTER 10
MANAGEMENT OF COGNITIVE AND PERCEPTUAL
DEFICITS
Orientation
Orientation refers to the awareness of self in relation to person,
place, time, and circumstance (Sohlberg & Mateer, 1989).
Orientation deficits are typically symptoms of brain dysfunction,
with disorientation to time and place being most common (Lezak,
1995).
Attention
The term attention was famously defined by William James as
“the taking possession by the mind, in a clear and vivid form, of
one out of what seem several simultaneously present objects or
trains of thought” (James, 1890, pp. 403–404). Attentional
abilities are dependent on multiple brain regions including the
cingulate cortex, limbic system, prefrontal cortices, and
59
sensorimotor regions (Posner, 1980). Each person is thought to
have a limited capacity for consciously attending to information a
hard-wired upper limit that dictates how many inputs can be
simultaneously processed (Lezak, 1995). Deficits in attention are
common following brain injury as numerous and diffuse neural
regions work to support the individual’s attentional abilities.
Deficits can also be experienced because of a lack of sleep or
decreased nutrition (Groeger et al., 2011). Learning is dependent
on attention as individuals can-not encode into memory stimuli
or content they do not attend to. Definition 6-1 describes five
components of attention (Sohlberg & Mateer, 2001) and their
implications for task performance.
Memory
Memory broadly refers to information storage and retrieval
(Lezak, 1995). Rather than a unitary process or construct, there
are many types of neural processes that support an individual’s
memory capacity (Sohlberg & Turkstra, 2011). Experts still
debate about what transpires during the process of remembering
(Cowan, 2008). Atkinson and Shiffrin’s (1971) Information-
Processing Model, which highlights stages of acquiring and
employing new knowledge and skills, is one of many conceptions
as to how this process occurs. We use this model to introduce
memory-related concepts, acknowledging the continued debate
regarding precise terms and their meanings in this realm. (Also
see Dubuc [2011] and Levy [2011] for more in-depth information
on the following discussion.)
Sensory Registers
Information from the environment is briefly (milliseconds) held in
registers (or stores) specific to the human senses (Lezak, 1995).
This registration stage has been called the intake valve for
determining what data from the environment are ultimately
stored. This phase is influenced by acuity of the senses (such as
hearing and vision), affective set, and perception.
Long-Term Memory
Whereas data in working memory have a short shelf life,
information in long-term memory can be stored for minutes to a
lifetime (Lezak, 1995). When we remember information (an event
that occurred an hour ago or a year ago), we have located and
retrieved data from long-term memory and are holding it for
conscious attention and thought in limited-capacity working
memory. Storage in long-term memory is based on relatively
permanent changes in brain cell structure (Glover, Ronning, &
Bruning, 1990), although there does not appear to be a single
local storage site for stored memories (Lezak, 1995). Long-term
memory is thought to consist of two subsystems, explicit (or
declarative) memory and implicit (or non declarative) memory
(Fig. 6-2). Declarative memory pertains to factual information and
includes episodic memory (knowledge of personal information
61
and events such as what you ate for breakfast) and semantic
memory (knowledge of facts about the world such as that horses
are big and ants are small) (Eysenck & Keane, 1990). Prospective
memory is another form of declarativememory, having to do with
remembering to do tasks that one intends (Fish, Wilson, &
Manly, 2010). Implicit or nondeclarative memory differs from
explicit memory because it does not involve conscious awareness
of learning (Sohlberg & Turkstra, 2011). This includes procedural
memory, which pertains to knowing how to do things; it allows us
to learn and perform skilled motor actions (Eysenck & Keane,
1990). Memory impairments are typically characterized as mild,
moderate, or severe based on the results of cognitive assessment.
The term neurologic amnesia is reserved to de-scribe losses of
broad categories or segments of memory resulting from brain
trauma, stroke, or tumor
Dynamic Assessment
Unlike static assessments, which identify and quantify
impairment at a specific point in time, dynamic assessment
refers to an approach in which the clinician uses cues and
feedback to understand how to elicit the patient’s best
performance (Toglia & Cermak, 2009). Dynamic Interactional
Assessment (DIA) (Toglia, 1998) is an example of a dynamic
assessment approach. DIA consists of awareness questioning,
cueing and task grading, and strategy investigation (Toglia,
1998). Patients predict their performance before beginning the
assessment task. Graded verbal cues are offered as needed once
the patient begins work, and parameters of the task are changed,
if necessary, to buoy the patient’s performance. The therapist
also asks questions about what strategies or approaches the
patient uses. Toglia (1993) incorpo-rated DIA into a number of
the standardized assessment tools she developed, including the
Contextual Memory Test. In addition to these specific measures,
therapists can convert any task (such as organizing the messy
62
cupboard in Fig. 6-3) into a dynamic assessment by deliberately
manipulating task and environment variables and offering
strategies and cues to determine in what conditions the patient
performs at his or her best (Dougherty & Radomski, 1993).
Informal Observation
Informal observation of task performance enables the therapist to
make hypotheses about cognitive strengths and weaknesses and
identify domains warranting further evaluation. For example,
during an ADL or homemaking evaluation, occupational
therapists observe attention to task by counting episodes of
distraction in a specific. Learning is a primary therapeutic
mechanism underlying many, if not all, occupational therapy
interventions. Occupational therapists teach patients to perform
activities of daily living (ADL), teach family members home
programs, teach other therapists new techniques, and teach the
public in community education courses. Each teaching
opportunity requires preparation: the physical environment
needs to be prepared; any needed materials, props, or equipment
needs to be gathered; and most importantly, the learner must be
pre-pared for the session. Teaching strategies need to be
consciously designed to maximize learning, using methods
designed to support different learning styles (e.g., visual, aural,
read/write, or kinesthetic learners) (Lofl and, 2009). Any
handouts should address health literacy concerns and are
developed with an understanding of the cultural, social, and
cognitive issues that might affect the teaching session (DeWalt et
al., 2010). All of these concerns need to be addressed in order for
effective teaching to take place. Finally as teachers, therapists
must determine whether learning has taken place. For example,
after a 30-minute dressing session, a patient who has had total
knee replacement surgery is able to don his pants, socks, and
shoes. From a traditional learning perspective, the patient’s
performance at the end of the session reflects the extent to which
he has learned the desired skill or strategy. However, what if a
nurse later reports that the patient is unable to carry out the
activities he demonstrated proficiency with the day before? One
can easily see that although within-session performance was
buoyed by cues and practice, true learning did not occur.
Schmidt and Lee’s (2011) contemporary definition of motor
learning allows us to differentiate learning from within-session
63
performance: “. . . motor learning is a set of processes associated
with practice or experience leading to relatively permanent
changes in the capability for responding” (p. 327). Based on this
definition of learning, we would expect the patient with knee
replacement to demonstrate similar levels of proficiency after the
occupational therapy session as during. The term performance is
used to describe what is seen during training, that is, short-term
capabilities resulting from instruction, cues, or assistance. For
occupational therapy to help patients resume occupational roles
therapists must understand the process of transforming
performance into learning and use teaching methods that help
the patient learn new skills. This chapter discusses the role of
information processing in learning and then summarizes the
array of variables that affect the patient-specific teaching plan.
Key influences on learning are detailed, and the chapter
concludes with specific applications to occupational therapy
practice. Regarding issues of learning, there has been a
traditional division in the research between motor and cognitive
tasks. In clinical practice, however, it is unusual to find motor
tasks that do not have a cognitive component or the reverse.
Because this distinction does not translate into practice and
because principles in the two areas are similar, the principles are
combined in this chapter.
64
CHAPTER 11
MOTOR LEARNING
Controlled Processing
Controlled processing is technically a temporary activation of a
series or sequence of elements in long-term memory under the
attention and control of the thinker (Schneider & Shiffrin, 1977).
For example, the new driver must actively recall specific rules
65
and instructions and direct his attention to each motor sequence
of the task. Con-trolled processing is limited by the capacity of
working memory and is therefore effortful but flexible in handling
novel situations. When learning a new task.
Processing
Thus, patients learning new skills or behaviors can process only
a limited number of inputs (instructions, cues, and
environmental distractions) at a time.
Automatic Processing
With enough controlled processing, a task requires less and less
concentration to carry out (Shiffrin & Schneider, 1977). That is,
the task becomes increasingly automatic, as in the example of
the proficient driver. Automatic processing occurs when specific
contextual stimuli internal or external to the person trigger the
activation of a specific learned sequence in long-term memory
(Schneider & Shiffrin, 1977). Given enough repetition, the
individual performs the skill or task in a consistent manner with
little or no attention. Sternberg (1986) suggested that
development of a full level of automatization requires at least 200
trials of a task but that automatization begins in as few as 10
trials, so long as those trials are consistent. With fully automatic
skills, people cannot stop themselves from per-forming the
overlearned sequence unless control processes are employed to
override it. Definition 13-1 discusses implications related to
helping patients with memory impairment to reacquire skills.
According to Giles (2005), overlearning is practice of a skill or
strategy well beyond demonstration of learning or proficiency; it
increases the likelihood that the skill or strategy will become
automatic. These automatic skills and strategies become the
easiest behaviors to initiate from an array of possible behaviors
(Giles, 2005), minimizing demands on attention and decision
making. Habits and routines are examples of automatic motor
sequences that, according to Kielhofner, Barris, and Watts
(1982), organize a person’s tasks, space and time. Characteristic
of automatic skills and strategies, habits and routines are
responsive to the environmental conditions under which they are
learned and develop with repetition (Kielhofner, Barris, & Watts,
1982; Wood, Quinn, & Kashy, 2002). Habit learning is a form of
implicit learning, which is described in Definition 13-2.
66
Occupational therapists often help patients resume or relearn
self-maintenance tasks so that they are once again automatic.
Learner Characteristics
A client’s learning potential is determined by many factors.
Variables affecting cognitive status (reviewed in Chapter 6) are
critical contributors to learning potential. Remember, these
considerations apply to family caregivers as well as patients (see
Procedures for Practice 13-1). Literacy is one important
dimension of the experiential sociocultural influence on learning,
with many implications regarding teaching methods (see Chapter
3). Neistadt (1995) suggested that occupational therapists
attempt to answer four questions regarding patients’ learning
capacities during the course of their traditional evaluation. These
questions include the following:
1. What modes of input (e.g., visual, auditory, and tactile) can
this patient process most easily?
2. What approaches to tasks (outputs or behaviors) are still
available to this patient?
3. What tasks remain meaningful or are most likely to facilitate
learning for this patient?
4. How well is this patient able to transfer learning, that is,
apply specific skills to a variety of tasks under
Reaction Time
One example of cognition and action that has been relatively well
researched as a measure of cognitive processing is reaction time.
Reaction time is considered to be a measure of how efficiently the
CNS is working. Reaction time is defined as the time from when a
stimulus is given to the time when a movement begins in reaction
to the stimulus. There are different types of reaction time. For
instance, simple reaction time is the reaction time when there is
a single stimulus associated with a solitary specific response. For
example, suppose that when a person sees a light turn on, he or
she is required to reach forward; in this case, there is a one-to-
one relationship between the stimulus and the response. Choice
reaction time is somewhat more complex, occurring when a
number of potential stimuli are present and the motor response
is unique for each of the different stimuli. For example, there
might be three potential stimuli involving a red light, a green
light, and a blue light. The directions might be that when he or
she sees the red light, the participant is required to lift his or her
arm straight up; when he or she sees the green light, the
participant is supposed to move his or her arm to the left; and
when he or she sees the blue light, the participant is supposed to
76
move his or her hand to the right. It has been shown that a
person’s choice reaction time performance is typically longer than
his or her simple reaction time performance. It has also been
shown that the best reaction times occur during the late teens
and early twenties and that the reaction time increases each
decade thereafter. Men tend to have faster reaction times than
women. It also appears that the more education a person has,
the better is his or her reaction time (Fozard, Vercryssen,
Reynolds, Hancock, & Quilter, 1994; Houx & Jolles 1993) Within
the individual movement is the product of many systems that
need to be examined as they interact and contribute to movement
organization and control. Deficits in one system affect the
function of other systems. To understand motor control and
organization, the perceptual, cognitive, and action components of
motor control must be considered and synthesized as part of the
full picture of motor control. In addition to individual body
factors affecting control, different tasks as well as the
environment impose constraints on motor control. The Person,
the Task, and the Environment Skill acquisition is dependent on
several factors. Practitioners help patients to learn skills, and
these skills have multiple dimensions that influence
reacquisition. Understanding the types and requirements of tasks
as well as the regulatory features of the environment that affect
task performance allows the practitioner to plan with the patient
the optimal environment for skill learning. Also, under- standing
how movement control is affected in the client with a neurological
disorder and what body systems have been affected helps the
practitioner to plan effective multi systems interventions for skill
learning. Along with using task analysis, a task taxonomy can be
useful for retraining functional movements in the patient with a
neurological disorder. Using the three continuums of closed to
open skills, stability to mobility, and no manipulation versus
manipulation, the practitioner could assess a client’s capability
and skill level across many basic and instrumental activities of
daily living. By systematically varying the regulatory features of
the task and the environment, the practitioner, can help the
patient to develop the motor program and plans that are
necessary for rebuilding task routines. Now that we have
reviewed some fundamental aspects of motor learning and
control and its development from a theoretical perspective, the
77
next section focuses on defining and assessing motor skills and
motor performance.
83
INTELLECTUAL HERITAGE AND OCCUPATIONAL THERAPY
THEORETICAL FRAMES OF REFERENCE GUIDING
NEUROMOTOR INTERVENTIONS
The field of motor control draws from a wide range of disciplines
and reflects the scholarly and research activities of scientists who
are interested in motor behavior. Research findings and theories
about motor control and recovery of function have had an
ongoing influence on the practice of occupational therapy focused
on adults and children with motor control disorders. In the years
following World War II, the dominant therapeutic approach that
was used in the field of physical disabilities was muscle
reeducation. This approach, although useful for polio, was not
appropriate for treating people with disorders of the CNS with
resultant paresis. Because of the inadequacies of applying
muscle reeducation principles to CNS disorders, a few
occupational and physical therapy practitioners (e.g., Ayers,
1972; Bobath, 1965; Brunnstrom, 1970; Knott, 1956; Rood,
1952; Voss, Ionata, & Myers, 1965) began to study and
hypothesize about how the nervous system controls movement
and applied these principles to clinical practice. As a result of
their efforts, the development of organized approaches and
techniques to restore CNS function emerged in the therapy fields.
The period from the mid-1950s through the 1980s was marked
by the development and teaching of specialized neurotherapeutic
approaches such as proprioceptive neuromuscular facilitation,
neurodevelopmental therapy, and Brunnstrom’s movement
therapy. These approaches were situated in the knowledge of the
time focused on specific sensorimotor techniques and
assumptions about the CNS and the organization of motor
behavior. More recently, these approaches have been less
favored, as there are more current theoretical approaches that
have better research evidence supporting their use. The field of
motor control and learning tries to explain both the regulation
and control of normal movements, as well as the factors and
processes that are involved in nor- mal motor learning. Motor
learning is commonly defined as the processes associated with
permanent changes in motor behavior resulting from practice
and experience. The reader is referred to texts that provide in-
depth explanations of the motor learning process and the
research that supports learning using different practice
conditions and the salient factors such as feedback that affects
84
motor learning (e.g., Brooks, 1986: Schmidt & Lee, 2005). The
motor control approaches that are used in occupational therapy
reflect an integration of ideas that explain the nature and
regulation of movement. There is no one singular motor control
theory of occupational therapy; rather, there are several applied
motor learning and control approaches and models. These
approaches are supported by motor learning and control research
drawn from the movement and therapeutic sciences and provide
evidence supporting different occupational therapy interventions.
Understanding motor control implies knowledge about what is
controlled and how the controlling processes are organized
(Horak, 1991). Motor control involves the ability to regulate or
direct the mechanisms that are essential to movement.
Rosenbaum (1991) has proposed that the central issues in motor
control revolve around the multiple factors that determine
movement selection, movement sequencing, and the coordination
of perception and action in goal-directed activities. For instance,
a fundamental question for motor control theorists is how
stability is maintained and controlled while the individual acts in
and on the environment. In the context of occupational
performance, this question becomes “How are postural stability
and movement regulated and controlled for in an individual
engaged in an everyday daily life activity such as dressing while
sitting on a stable or unstable surface such as a chair or soft
mattress?” Motor learning is directed more toward understanding
how movements are acquired and modified with practice.
Schmidt (1988) has defined motor learning as a set of processes
associated with practice or experience leading to permanent
changes in the capability for skilled acts. Shumway-Cook and
Woollacott (2001) have proposed that motor learning develops
from a complex set of perceptual, cognitive, and action that are
processes developed in response to individual-task-environment
interactions. The field of motor control and learning continues to
provide occupational therapy with new ideas for under- standing
the nature, cause, acquisition, and modification of movement
supporting optimal occupational performance. The following
section provides a synopsis of the prevailing motor learning and
control theories and their implications for occupational therapy
treatment. Theories are organized according to whether the
control is centralized within the CNS or dispersed throughout the
CNS and or other systems.Motor development is the culmination
85
of a number of underlying subsystems. These underlying
subsystems all develop and mature at their own rates relative to
other sub- systems. Examples of these subsystems include, but
are not limited to, the skeletal system, the muscular system, the
central and peripheral nervous systems, the endocrine sys- tem,
and the sensory systems (visual, auditory, gustatory, olfactory,
haptic, proprioceptive, and vestibular systems). Historically,
motor development was thought to occur through specific and
prescribed stages. Gessell (1928) and others (Gessell & Ames,
1947; McGraw, 1935) developed. ontogenetic stages that depict
developmental milestones for children from approximately 6
months to 6 years of age. These developmental milestones
include postural and motor abilities that are believed to be
stereotypical of various stages of development. The typical
development follows certain directions, specifically cephalocaudal
(head to tail) and proximodistal (axial to extremities). In other
words, developmentally, a child gains control of the head before
gaining control of the lower parts of the body (e.g. the ability to
walk). In a similar vein, developmental milestones show that
proximal stability is required before the affordance of distal
mobility. If the trunk is not stable, the ability to successfully
reach for an object will be diminished. These directionally
dependent constructs require that the CNS organization is largely
hierarchical in nature. That is, higher, more complex parts of the
brain control or have some dominion over lower centers of the
brain. Most primitive reflexes have their neuroanatomical origin
in the midbrain, cerebellum, and medulla. As was stated earlier,
if damage occurs to a higher area of the brain (e.g., the cerebral
cortex), then the reemergence of primitive reflexes is not
uncommon. This gave empirical evidence that reaching
developmental milestones within the “normal” stereotypical time
periods was a good indication of the child’s overall development.
Further, the evaluation and assessment of primitive reflexes and
developmental milestones was believed to be a reliable method for
evaluating the potential for success in age appropriate
occupations (Capute et al., 1982). Recently, however, there has
been some question as to the veracity of this assumption
(Bartlett, 1997). Bartlett evaluated 156 infants who were believed
to be developing normally on the Primitive Reflex Profile and the
Alberta Infant Motor Scale. Assessments were at 6 weeks and at
3 and 5 months. Bartlett found no statistical correlation between
86
the developmental scale scores and the primitive reflex scores.
Thus, Bartlett concluded that there was no relationship between
motor development and the presence of primitive reflexes. Along
the same lines, Thelen (1986) found that when a 6- to 7-month-
old infant was supported over a treadmill, the infant
demonstrated a relatively mature bipedal stepping motion. This is
particularly interesting because the traditionally held ontogeny of
developmental milestones asserted that the stepping motion
normally appears as a newborn but then dis- appears at
approximately 2 months only to reappear at approximately 12
months of age (Strauss, 1982). What Thelen has shown was that
when the environment was manipulated (e.g., by supporting the
child’s body weight, thereby reducing the effects of gravity), the
child spontaneously demonstrated a precocious bipedal stepping
motion. Because it is illogical to think that the CNS matured only
while the child was supported, it is clear that the assumed
hierarchical organization of the CNS is not the exclusive factor or
control mechanism in motor development. Another line of
evidence regarding grip configuration questions the traditionally
held belief that there is a stereotypical sequence of hand grasp
configurations (J. S. Connolly & Elliott, 1972; K. J. Connolly,
1973; Gessell, 1928). This sequence involves the initial use of a
gross grasp/ clawlike configuration and ending up with a mature
finger thumb opposition configuration. One group of researchers
(Newell, Scully, Tenenbaum, & Hardiman, 1989) developed an
interesting experiment in which the participants were asked to
grasp blocks of varying sizes from 0.8 cm to 24.2 cm in width.
Participants included 26 preschoolers aged 3 years 3 months to 5
years 4 months and 22 adults aged 18 to 46 years. These
researchers found that when the ratio of the width of the block to
the size of the hand was the same, similar types of grip
configurations were elicited regardless of the age of the
participant. These results demonstrated that if the child’s
environmental constraints are manipulated to match the
constraints that an adult would face, the child will demonstrate
grip configurations similar to those that the adult employed.
Subsequent studies have shown similar results (Cesari & Newell,
2000; Newell, McDonald, & Baillargeon, 1993; Newell, Scully,
McDonald, & Baillargeon, 1989). Conceptually, these studies by
Thelen, Newell, and others offer a departure from the belief that
development is based on hierarchical organization of the CNS. On
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the basis of theoretical work by Bernstein (1967), these more
contemporary theorists believe that the “system” (meaning the
various organ, tissue, and bodily systems) is organized in a
heterarchical fashion. This means that the method by which a
person successfully plans and executes a motion requires the
input of many subsystems (e.g., muscle groups, nervous
systems), each of which contributes to the movement solution as
the task requires. The subsystems are not necessarily under the
direct control of the cerebral cortex; that is, the cerebral cortex or
consciousness regarding the motor plan does not prescribe the
specific details of what each subsystem must do for a successful
motor action. Bernstein (1967) argued that although the
“executive function” knows the general goal of the task at hand, it
does not have the capability of knowing the precise myriad of
details required by each of the subsystems. The executive
function knows the goal of the task and can direct in a very
general manner, but the subsystem is what handles the small
details, such as how much range of motion is required at each
joint, how much force is required with each muscle, and how
many motor neurons to recruit, the sequential timing of the
agonist and antagonist muscles. Bernstein (1967) further argued
that the subsystems tend to work together in synergistic
patterns. For example, suppose that a person is sitting on a sofa
and reaches for a television remote control that is two feet in
front of the person on top of a coffee table. Certain muscle groups
are recruited, such as the anterior deltoids and the triceps, as
the primary muscles to fling the arm forward to the remote. Now
suppose that the person is lying on his or her side on the sofa
instead of sitting. The person reaches for the remote as in the
previous example, but because the position of the arm in relation
to the remote and to gravity is different, a different set of primary
muscles is recruited (namely, the medial deltoid to counteract the
direct pull of gravity, along with the anterior deltoid and the
triceps) to move the arm in the direction of the remote. If one
were to compare the electrical activity of the anterior deltoid and
triceps between the two conditions, the electromyography would
be completely different. An important thing to remember in this
example is that although the goal was the same in both
situations (i.e., grab the remote), different subsystems were
recruited depending on what the situation required. The following
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section provides more information about some specific models of
motor control that take these concepts into account.
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MOTOR CONTROL APPROACHES PROPOSED FOR
THERAPEUTIC INTERACTION
New approaches to intervening with motor performance deficits
that affect occupational performances have evolved. These
therapeutic approaches are based on both motor learning
principles and on more contemporaneous models and research
about the control of movements in both typical populations and
populations with movement disorders. Task-Oriented Model The
task-oriented model (Gordon, 1987; Horak, 1991; Shumway Cook
& Woollacott, 2001) targets both peripheral and central control
systems. In line with system models of motor control, the task-
oriented model assumes that control of movement is organized
around goal- directed functional tasks. Clients are taught to
accomplish goals for functional tasks. By practicing a wide
variety of movements, the client solves different types of motor
problems. The assumptions seen in Table 55.6 guide treatment.
Along with these assumptions and guidelines, Horak (1991)
suggests organizing questions around several areas in treating
clients with motor performance deficits. These areas are the
client’s behavioral goals, movement strategies, musculoskeletal
constraints, compensatory strategies, and need for adaptations.
Examples of questions about these areas are as follows:
1. Behavioral goals: Are the therapist’s and client’s goals the
same? This might entail the practitioner incorporating the
use of the Canadian Occupational Performance Measure
(COPM) and the Assessment of Motor and Process Skills.
Using both would allow the practitioner to determine what
is important to the client and what are the strengths and
weaknesses in the client’s motor and process skills.
2. Movement strategy: What are the organizing principles of a
normal movement strategy?
3. Musculoskeletal constraints: How much of the motor
deficit in a patient with neurological deficits is due to a
deficit in the musculoskeletal system rather the neural
components?
4. Compensatory strategies: Has the patient found the most
effective strategy?
5. Adaptation: How must a movement strategy be adapted to
accomplish a task in a new environmental context?
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CHAPTER 12
CARR AND SHEPHERD
MOTOR RE-LEARNING PROGRAM
93
performance emerges from the interaction between personal
characteristics and performance contexts.
94
over the side of the bed, balanced sitting, and standing up
and sitting down, balanced standing, and walking.
The Task-Oriented Approach (Mathiowetz, 2004) considers the
person’s role performance as well as the performance in areas of
occupation. In addition, this model espouses that a task analysis
be performed to determine the factors (from the person, the
environmental context, and the task itself, the same three factors
as in the perceptual motor workspace) that either facilitate or
restrict performance. The treatment focus of this approach
involves any individual or combination of the following:
Environmental modification
Manipulating or grading the task
Remediating skills/abilities that are particularly limiting
within the person
Implications
Specific strategies are espoused for the remediation of limiting
factors within the person. Many of these strategies are founded in
the motor learning body of knowledge. Some of these strategies
involve how feedback is given. For instance, it has been shown
(particularly with nondisabled populations) that reduced
feedback actually results in better retention and transfer of the
motor skill being learned (Rice, 2003; Rice & Hernandez, 2006;
Winstein & Schmidt, 1990; Wulf, Schmidt, & Deubel, 1993). See
Shea and Wulf (2005) for a review. Another motor learning
strategy involves presenting tasks to be learned or practiced in a
random fashion (e.g., moving from task to task without providing
repeated trials on any given task) rather than giving multiple
trials of the same task before moving on to another task.
Limitations Much of the motor learning research has been
performed on healthy college students, and the tasks have
usually involved simple rote motor skills that do not represent
normal occupationally oriented activities. Therefore, the
generalization of these strategies to special populations should be
done with caution until a greater body of evidence supports their
use with special populations.
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Functional tasks help to organize behavior. Recent research
suggests that parameters of motor behavior are not
performance components but in fact functional goals
(Burton & Davis, 1992; Gentile, 1992; Heriza, 1991; Thelen,
1989).
Occupational performance emerges from the interaction of
multiple systems that represent the unique characteristics
of the person and the performance context.
After CNS damage or other changes in personal or
environmental systems, clients’ behavioral changes reflect
their attempts to compensate and achieve functional goals.
Personal and environmental systems are hierarchically
organized. There is no inherent ordering of the personal and
environmental systems in terms of their influence on motor
behavior. There is also no inherent ordering within the
system, even within the CNS.
A person must practice and experiment with varied
strategies to find optimal solutions for motor problems and
develop skill in performance.
Because the primary purpose of motor behavior is to
achieve functional goals, therapists begin and end therapy
by focusing on occupational performance. The emphasis on
task performance and evaluation is primarily at the
disability level, using the World Health (1980) Organization
Model of Disablement.
The therapist assesses all systems that are contributing to
problems in functional performance or supporting optimal
performance, keeping in mind the tasks the person
currently does or will be doing in the future. Because the
client brings to the situation a unique constellation of
characteristics, the therapist makes the client’s perspective
the focus of assessment. The client determines the
important goals and roles necessary for occupational
performance.
Movement patterns that are used for compensation and
achievement of functional goals must be understood fully.
The evaluation of occupational performance must include
an examination of the process (actual movement patterns),
the outcome, and the stability or instability of observed
motor behavior.
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Evaluation strategies consider all personal and
environmental systems. The ones that interfere the most
with performance are evaluated first.
As part of treatment, clients practice, experiment, and
problem-solve in order to achieve functional goals.
Treatment planning is to develop and implement learning
opportunities for clients with problem-solving abilities.
When clients are unable to problem solve, the therapist
might need to train them to use given routines.
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CHAPTER 13
COGNITIVE IMPAIRMENTS: DEFINITIONS,
EVALUATIONS, AND INTERVENTIONS
Self-Awareness
Impaired self-awareness associated with neurological dysfunction
includes lack of knowledge about one own physical or cognitive-
perceptual impairments and/or their functional implications as
well as inability to anticipate difficulties, recognize errors, or
monitor performance within the context of an activity (Toglia &
Kirk, 2000). Impaired self-awareness presents obstacles to
adjustment, collaborative goal setting, and active participation in
intervention. Decreased awareness results in poor motivation and
compliance, lack of sustained effort, unrealistic expectations,
incongruence between goals of the client and family, impaired
judgment and safety, and inability to adopt use of compensatory
strategies (Hartman-Maeir, Soroker, Oman, & Katz, 2003; Sherer,
Oden, Bergloff, Levin, & High, 1998; Toglia & Kirk, 2000). A
number of studies support the association between awareness
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and functional outcome (Fischer, Gauggel, & Trexler, 2004;
Goverover, 2004; Hoofien, Gilboa, Vakil, & Barak, 2004; Noe et
al., 2005; Tham, Ginsberg, Fisher, & Tegner, 2001). Unawareness
may be related to psychological or neurological sources. Denial is
a psychological defense mechanism that is related to premorbid
personality traits and is characterized by over rationalization,
hostility, resistance to feedback, and an unwillingness to
confront problems (Prigatano, 1999). A person who has a history
of denying inadequacies and resisting help from others and a
strong desire to be “in control” is more likely to use denial as a
coping strategy. Impaired self-awareness resulting from neuro-
logical lesions, represents a lack of access to information
regarding one’s cognitive state, and is characterized by surprise,
indifference, or perplexity in response to feedback (Prigatano,
1999). In many cases, the neurological and psychological sources
of unawareness coexist and cannot be easily differentiated. If
denial is the predominant source of unawareness, methods of
awareness training might not be effective (Lucas & Fleming,
2005).
Orientation
Orientation is the ability to understand the self and the
relationship between the self and the past and present
environment. Orientation depends on the integration of several
mental activities that are represented in different areas of the
brain. Disorientation is indicative of significant impairments in
attention and memory (Lezak, Howieson, & Loring, 2004). For
example, disoriented clients might think they are home rather
than in a hospital, might confuse the hospital staff with relatives,
or might believe it that is a new day each time they wake up from
a short nap.
Evaluation
Evaluation of orientation traditionally includes the client’s
orientation to person, place, and time. Orientation to person
involves both the self and others. Is the client able to report
personal facts and events and describe his or her previous
lifestyle? Does the client recognize people and associate them
with their role and name? Orientation to place is demonstrated
by the client’s ability to understand the type of place he or she is
in (e.g., a hospital), to report the name and location of the place,
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and to appreciate distance and direction. Orientation to time
requires an ability to report the current point in time (e.g., day,
month, and year), to show understanding of the continuity and
sequence of time (i.e., estimation), and to associate events with
time. Topographical orientation, often considered a component of
orientation to place, is the ability to follow a familiar route or a
new route once given an opportunity to become familiar with it.
Functionally, the person might not be able to find his or her way
from the therapy area to his or her room or describe and draw
the layout of a familiar room or route (Unsworth, 2007).
Difficulties with the visual-spatial and memory aspects of
topographical orientation need to be distinguished during
evaluation (Brunsdon, Nickels, & Coltheart, 2007; Unsworth,
2007). Orientation assessments are traditionally covered in
mental status examinations. occupational therapists frequently
use non-standardized measures of orientation, such as
interviews with open ended questions asked in a conversational
or informal manner. Most practitioners use cues to determine the
severity of the disorientation. If the client is unable to answer the
questions independently, the practitioner might offer a multiple-
choice array or verbal cues. Cues usually move from general or
abstract to more concrete, as deter- mined by the severity of
disorientation (e.g., “Today is the beginning of the work week”
versus “Today is the day after Sunday”). The number and type of
cues offer a method for scoring and monitoring progress.
Fluctuations in orientation during the day should be noted, as
clients might experience sundowning, in which they become
confused in the evening because of fatigue.
INTERVENTION
STRATEGY TRAINING AND/OR ADAPTATIONS OF TASK OR
ENVIRONMENT
Strategy training for disorientation involves teaching the person
to look for external cues when he or she is feeling confused or is
having difficulty recalling orientation information. For example,
an information poster that contains orientation facts can be
placed on a wall, in a closet, or eventually inside a notebook.
When the client is asked orientation information, he or she is
expected to locate the information poster to verify responses or to
find the correct answers. A memory book, containing pictures
and names of familiar people or important life events, can also be
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placed in a key location within the room. As an alternative, an
audiotape or videotape can be created by a family member to
review orientation information at set times during the day or
used whenever the person feels confused. An alarm that is
preprogrammed to ring several times a day can be used to cue
the person to read his or her orientation fact book or listen to the
audiotape. Orientation questions with use of cueing strategies
can also be incorporated into a bean bag toss game, a board
game, or a “Family Feud” style game within a group format
(Toglia & Golisz, 1990). A calendar posted on the wall or closet
may be helpful in orienting the person to time. If the client has
poor selective attention, a single piece of paper with the day and
date written daily, rather than a monthly calendar, might be
needed. To assist the client in finding his or her room, directional
arrows can be placed in the hallway, and tape indicating the
route to his or her room can be placed on the floor. Key
landmarks can be pointed out and made more salient with
arrows or colored tape. The therapist needs to immediately
reinforce initiation or use of any of these external cues by
praising the client (or rewarding points), and each time the client
initiates the use of an external cue, the therapist should keep
track of it by recording it on a chart or visual graph. The use of
external cues should be gradually faded until the orientation
information is internalized. In addition, the person should be
trained to look for orientation cues (e.g., clocks, calendars) in
different environments. Spaced retrieval techniques can be used
to train use of strategies and external aids, such as using a daily
calendar. Spaced retrieval involves systematically lengthening the
period of retention and recall. There is evidence that this
technique is more effective than cueing hierarchies in treating
people with dementia (Bourgeois et al., 2003).
Attention
Attention is a multidimensional capacity that involves several
components:
Unilateral Neglect
Unilateral neglect is a failure to orient to, respond to, or report
stimuli that are presented on the side contralateral to the
cerebral lesion in clients who do not have primary sensory or
motor impairments (Heilman, Watson, & Valenstein, 2003). The
term neglect connotes a volitional component to the disorder, but
this is a misnomer. The client with unilateral neglect is unaware
of the incompleteness of his or her perception of, and responses
to, the environment. He or she often behaves as though one half
of the world does not exist (Corben & Unsworth, 1999). For
example, following right-hemisphere strokes, clients often begin
scanning on the right side and miss or fail to explore most of the
stimuli on the left. Asymmetry may be observed in functional
activities, drawing tasks, reading, or writing. In severe cases,
clients may eat food on one side of their plate, shave half their
face, or dress half of their body without recognizing that anything
is wrong. In milder cases, they may misread the first letter of a
particular word or fail to attend to information while crossing a
street, shopping, or driving (see the Ethical Dilemma). Many
clients with unilateral neglect also exhibit anxiety or flattened
affect. Unilateral neglect has been identified as a major factor
impeding functional recovery in clients who have sustained
strokes (Chen Sea, Henderson, & Cermack, 1993; Cherney,
Halper, Kwasnica, Harvey, & Zhang, 2001). Those with unilateral
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neglect have more difficulty resuming activities of daily living,
have longer hospital stays (Gillen, Tennen, & McGee, 2005; Katz
et al., 1999), and are at increased risk for accidents (Webster et
al., 1995). Unilateral neglect has been described as a
heterogeneous disorder that includes different clinical subtypes
and behavioral components (Mesulam, 1994; Pierce & Buxbaum,
2002; Stone, Halligan, Marshall, & Greenwood, 1998). Unilateral
neglect can involve one or more modalities, may vary with the
nature of the stimuli (e.g., verbal versus nonverbal), and can
encompass single objects or different spatial frames of space:
extra personal or large space, peri personal or space within
reach, and personal or body space (Mesulam, 2000; Plummer,
Morris, & Dunai, 2003). For example, some clients demonstrate
neglect symptoms in large spaces, such as a room (extrapersonal
neglect), but do not have reduced awareness of their body
(personal neglect) or difficulty on paper-and-pencil tasks
(peripersonal neglect). Neglect subtypes have also been proposed
that involve internal mental images (representational neglect),
decreased movement into or toward the contralesional space
(motor neglect), or decreased ability to perceive sensory stimuli in
contralesional space (sensory neglect) (Mesulam, 1994).
Evaluation
Occupational therapists evaluating clients with unilateral neglect
must first distinguish between hemianopsia and unilateral
neglect. Visual field cuts (hemianopsia) are hemiretinal, while
neglect is hemispatial. Clients with visual field cuts typically have
awareness of their visual field loss and make compensatory head
movements and turns. Unilateral neglect may exist with or
without hemianopsia, and one syndrome does not cause the
other. Assessment of unilateral neglect typically involves
cancellation tasks that require detection of target stimuli,
distributed on both sides of space (see Table 57.1). Typically, the
majority of targets on the contralesional side of space are missed.
The complexity of unilateral neglect symptoms is not fully
captured by traditional tests of neglect. Therefore, it is important
not to rely completely on test instruments in identifying
unilateral neglect. The different behavioral manifestations and
subtypes of neglect need to be kept in mind during observation of
performance (Appelros, Nydevik, Karlsson, Thorwalls, & Seiger,
2003; Plummer et al., 2003). Dynamic assessment of unilateral
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neglect provides information about task conditions that increase
or decrease the symptoms of unilateral neglect as well as the
person’s ability to respond to different types of cues or implement
and carryover learned strategies to different situations. Toglia
(2005) has described a dynamic object search task that analyzes
the ability to learn and apply a strategy across a series of search
tasks in people with unilateral neglect.
Intervention
Specific skill training is emphasized in this section because
evidence exists to support the use of visual scanning training to
remediate disorders of unilateral neglect. In unilateral neglect,
clients demonstrate decreased eye movements to the affected
side. This decrease in eye movements reflects a decrease in
attention to one side of the environment (Antonucci et al., 1995;
Toglia, 1991b). A scientific literature review by Cicerone and
colleagues (2000, 2005) concluded that there is level 1 evidence
to support use of visuospatial interventions that include practice
in visual scanning because it improves compensation for
unilateral neglect and generalizes to everyday activities.
Therefore, they recommended visuospatial rehabilitation with
visual scanning as a practice standard for clients with visual
neglect after right-hemisphere stroke. The combination of forced
limb activation or movements of the left arm or hand on the left
side of space in conjunction with visual scanning also shows
positive results (Cicerone et al., 2005; Robertson, Hogg, &
McMillan, 1998). Intervention appears to be most effective when
a wide combination of intervention activities, including everyday
tasks, is used (Antonucci et al., 1995; Pizzamiglio et al., 1992).
Programs with greater levels of intensity have generally produced
more positive outcomes. However, even with intensive training, it
has been demonstrated that people with unilateral neglect have
poorer functional outcome than do other people with stroke
(Paolucci, Antonucci, Grasso, & Pizzamiglio, 2001). Weinberg and
colleagues (1977) designed systematic training techniques that
incorporated a combination of remedial worksheets and strategy
training techniques during reading and scanning tasks. For
example, they used graded anchoring, pacing the speed of
scanning, feedback, and decreasing the density of the stimulus.
Anchoring, or teaching the person to use a spatial reference
point, such as a colored line on the left side, is a common
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strategy in visual scanning training. Gross motor activities
involving vestibular input and whole body movement in space
increase general arousal and alertness and have been used in
combination with visual scanning activities to increase gaze and
attention to the affected side (Cappa, Sterzi, Vallar, & Bisiach,
1987). Activities such as balloon volleyball, with the client hitting
the balloon with his or her hands clasped together, is an example
of such an activity. Other intervention techniques that have been
recommended for clients with unilateral neglect include use of
prisms and visual occlusion techniques (Pierce & Buxbaum,
2002). Prisms cause an optical deviation of the visual field to the
right so that objects appear to be moved farther to the right than
they actually are (Redding & Wallace, 2006). Partial visual
occlusion methods attempt to force the person to use the
neglected visual field by patching the eye ipsilateral to the lesion,
patching the non-neglected half field of eyeglasses (Beis, Andre,
Baumgarten, & Challier, 1999), or darkening the nonneglected
half field of eyeglasses (hemispatial sunglasses) (Arai, Ohi,
Sasaki, Nobuto, & Tanaka, 1997). Recently, computer-assisted
training programs for street crossing and wheelchair navigation
have been described. Trained subjects with unilateral neglect
performed better on real-life tasks after virtual reality training
than control subjects did. The use of virtual reality–based
technology appears to show potential for clients with unilateral
neglect (Katz et al., 2005; Webster et al., 2001).
STRATEGY TRAINING
Strategies for unilateral neglect can be practiced within everyday
tasks such as setting a table for several people, dealing a deck of
cards to six people, identifying appointments on a wall calendar,
reading a newspaper, addressing envelopes of different sizes, or
identifying all the pictures or chairs in the room. Because
unilateral neglect symptoms vary with the size of space,
arrangement of space, and amount and density of information
presented, these activity parameters need to be matched with the
neglect symptoms and systematically varied and graded in
treatment. In some cases, treatment activities should emphasize
large-space activities; in other situations, activities should focus
on tabletop tasks that involve visual detail. In general, activities
that are unpredictable or involve stimuli randomly scattered on a
table or page are more sensitive to the symptoms of unilateral
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neglect than are activities that are arranged in a predictable,
structured, or horizontal array (Ferber & Karnath, 2001).
Intervention should include practice in identifying situations in
which neglect symptoms are most likely to occur, such as filling
multiple bowls with salad, placing cookie dough on a baking
sheet, or arranging photographs in a picture album. Individuals
with unilateral neglect do not always know when they are
attending to the left side. Intervention needs to assist clients in
finding external cues that will provide feedback about when they
are indeed attending to the left. An emphasis in intervention
should be teaching the client to find the edges of a page or a table
or the periphery of stimuli before beginning a task and to mark it
with spatial point of reference, such as colored tape, a colored
highlighter, a bright object, or placement of his or her arm on the
left border. Auditory cueing, utilizing a beeper or alarm device,
can be combined with strategy training to remind the person to
use a strategy or visual cue. The alarm device can require the
client to scan space and attend to the left to turn off the sound
(Seron, Deloche, & Coyette 1989). Other intervention strategies
for unilateral neglect include tactile search, use of mental
imagery, and general alerting techniques. Tactile search includes
teaching the client to feel the left side of space with eyes closed or
to feel the left edges of objects before visual search. Visual
imagery teaches imagining and describing familiar scenes or
routes and using mental images during movement of limbs or
visual scanning (Niemeier, 1998; Smania, Bazoli, Piva, &
Guidetti, 1997). For example, reduction in neglect symptoms and
increased performance on functional tasks were reported after a
mental imagery program that involved teaching people with
neglect to imagine their eyes as sweeping beams of a lighthouse
from left to right across the visual field. Clients were cued to use
this mental image during functional and therapy training tasks
(Niemeier, 1998; Niemeier, Cifu, & Kishore, 2001). In addition to
strategies specifically aimed at facilitating attention to the left
side, strategies that focus on the general ability to sustain
attention have also been found to reduce unilateral neglect. For
example, Robertson, Tegner, Tham, Lo, and Smith (1995) taught
clients with chronic unilateral neglect to mentally tell themselves
to “pay attention” and to tap loudly on a table. It has been
observed that response to strategy training depends on whether
people with unilateral neglect show improvements in their
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awareness (Tham et al., 2001; Robertson & Halligan, 1999). This
underscores the importance of deeply embedding awareness
training techniques, such as those described earlier, into all
intervention activities.
Visual Processing
Visual perception is viewed on an information-processing
continuum involving the reception, organization, and assimilation
of visual information. On one end of the continuum, simple
visual-processing tasks such as matching shapes or objects
occur quickly and automatically, with minimal effort. On the
opposite end of the continuum, complex visual tasks that include
unfamiliar stimuli or subtle discriminations within visually
crowded arrays require slower and effortful processing. In this
conceptualization, visual-processing dysfunction is defined as a
decrease in the amount that the visual system is able to
assimilate at any one time (Toglia, 1989). To understand the
client’s visual perceptual skills and the effects of impairments on
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functioning, we need to analyze the activity conditions
(complexity, amount, familiarity, and predictability) rather than
the type of activity (visual spatial, visual discrimination, visual
motor, or visual gestalt). Problems in simple visual processing
include difficulty in discriminating between objects, pictures of
objects, and basic shapes; difficulty in detecting gross differences
in size, position, direction, angles, and rotations; decreased
ability to visually locate single visual targets in space or judge
gross distance between two objects; and decreased ability to
detect simple part-whole relationships in objects or basic shapes.
The person may have difficulty in familiar and routine activities
and may easily misinterpret or misidentify objects. Failure to
recognize an object is labeled visual agnosia. Toglia (1989)
proposes that labels such as visual agnosia are too broad for the
purposes of intervention because there are many different
underlying reasons for object recognition difficulties. For
example, a person might fail to attend to the critical feature of an
object or the part of the object that tells what it is (e.g., prongs of
a fork). Attention might be captured by salient but irrelevant
aspects of the object (e.g., the utensil’s decorative handle). There
might be an inability to process the overall shape and the details
simultaneously, so the person might miss important details.
Complex visual processing skills are required in visually
confusing environments; when there is abstract, unfamiliar, or
detailed visual information; or in conditions under which the
distinctive visual features are partially obscured (e.g., the object
is rotated and partially hidden on a crowded desk). Dysfunction
of complex visual perceptual skills may include decreased ability
to detect subtle differences in abstract shapes and objects or
angles, size, distance, and position. A client might have difficulty
making sense out of ambiguous, incomplete, fragmented, or
distorted visual stimuli. The client might misinterpret an object
when it is in an unusual position or partially hidden. The person
might experience increased difficulty in visually confusing or
crowded environments. Functional tasks such as finding items in
a crowded closet, drawer, desk, or supermarket shelf and locating
key information on a bill, map, or schedule might present
difficulty. On these tasks, the person might misinterpret
information, Miss key visual details, or become sidetracked by
irrelevant visual stimuli.
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Visual Motor
Visual motor skills include drawing tasks (e.g., drawing a map,
copying a design) or construction of three- dimensional figures
(e.g., assembling a coffeepot). Clients may demonstrate difficulty
on visual motor tasks for many reasons. For example, a client
might have difficulty constructing a block design because of a
poor ability to scan the complete design, decreased planning and
organization, unilateral neglect, or impaired discrimination of
size, angles, and rotations. The term constructional apraxia is
used to refer to difficulty with drawing or assembly tasks that
cannot be attributed to primary motor or sensory impairment,
ideomotor apraxia, or general cognitive impairments (Farah,
2003). Constructional abilities are closely related to ADL
performance (Neistadt, 1992a; Warren, 1981). Clients may have
difficulty dressing (dressing apraxia), orienting clothes correctly
on a hanger, or assembling a sandwich or coffeepot. People with
left-hemisphere parietal lesions tend to omit individual pieces or
details in constructional tasks, whereas those with right-
hemisphere lesions demonstrate spatial disorganization of the
pieces and lose the overall gestalt (Kramer, Kaplan, & Blusewicz,
1991). Constructional apraxia is not a unitary syndrome.
Impairments in different types of perceptual processing or spatial
relations are thought to underlie constructional apraxia in both
right and left-hemisphere lesions (Laeng, 2006).
Evaluation
Evaluation for people with visual perceptual impairments should
examine visual foundations skills, visual abilities without a motor
response, and visual motor skills. Visual foundation skills,
including visual acuity, oculomotor skills, and visual fields,
should be evaluated prior to a visual processing evaluation to
screen out visual problems that will interfere with the accuracy of
perceptual testing (Cate & Richards, 2000). Several clinical
observations during functional tasks can alert occupational
therapists to the need for a formal visual assessment:
compensatory head movements and tilting, squinting, shutting of
one eye, or a tendency to lose one’s place while reading. A basic
screening can be performed by the occupational therapist (i.e.,
visual acuity, range of motion of the eyes, ocular alignment,
visual pursuits or smooth tracking of moving objects, saccades or
quick eye movements to place an object of interest in view, and
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visual-scanning functions). Any disruptions of these foundational
skills will affect interpretations of higher level visual-processing
assessments (Warren, 1993). Standardized nonmotor
assessments of visual perception (see Table 57.1) categorize
visual perception into specific skills such as figure-ground,
position in space, form constancy, spatial relations, and visual
recognition. Adults with neurological lesions may have difficulty
performing various types of visual processing tasks for similar
reasons (e.g., a tendency to over focus on parts, a tendency to
miss visual details, failure to simultaneously attend to the details
as well as the whole). Therefore, Toglia (1989) recommends an
approach that conceptualizes visual processing on a continuum
and evaluates both conventional and unconventional objects
under a variety of different activity conditions. In a dynamic
approach to visual perception, the therapist systematically
manipulates activity parameters and analyzes responses to cues
to understand why a client is having difficulty accurately
discriminating objects or visual stimuli (Kline, 2000; Toglia,
1989; Toglia & Finkelstein, 1991). Visual perceptual assessment
should examine responses to activities with and without a motor
response to examine differences in performance. Visual motor
skills are typically evaluated with block designs, puzzles, or
copying designs. The therapist needs to observe how the person
begins and how he or she proceeds. For example, does the client
begin by drawing the details rather than attending to the overall
shape of the figure? Informal observations in tasks such as
copying a map route, assembling a coffeepot or woodworking
project, wrapping a package, packing a lunchbox, or folding
clothes can provide additional information on visual motor
abilities. Symptoms may include angular deviations; improper
position, location, spacing, or alignment of parts; and spatial
distortions. The client’s ability to recognize and correct errors in
alignment or position should be investigated. For example, some
clients do not recognize visual spatial errors even when attention
is directed to the problem area, whereas other clients recognize
errors but are unable to correct them.
Intervention
Interventions may address visual foundations skills or visual
processing skills with or without a motor response.
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VISUAL FOUNDATION SKILLS
Treatment of visual foundation skills such as visual acuity and
contrast sensitivity, oculomotor skills, and visual fields generally
involves adaptations a such as large-print reading materials;
magnifiers; talking devices; increasing contrast of edges, borders,
or backgrounds; and changes in lighting. However, remedial
exercises may be recommended for individuals with oculomotor
or visual field deficits. For example, range-of-motion eye exercises
to the involved muscle have been advocated for individuals with
eye muscle pare- sis. Occlusion of the intact visual field with eye
patching has been used to force use of the impaired visual field
(Warren, 1993).
STRATEGY TRAINING
Strategies that maximize the client’s ability to process visual
information can be trained within everyday activities that involve
choosing among objects that are similar in shape and size (e.g.,
matching socks, sorting teaspoons and soupspoons); locating
information within supermarket circulars, calendars, maps, or
schedules; arranging information within grids or spread- sheets;
copying patterns in arts and craft activities; or finding
information in crowded draws, shelves, tables, or bulletin boards.
Strategies can include getting a sense of the whole before looking
at the parts; teaching the person to partition space before
localizing details; using one’s finger to scan, trace visual stimuli,
or focus on details; covering or blocking visual stimuli when too
much information is presented at once; verbalizing salient visual
features or subtle differences; and mentally visualizing a
particular item before looking for it (Toglia, 1989, 1998).
Intervention involves careful manipulation of activity parameters.
Activities that involve familiar items or contexts, high contrast
(e.g., red socks and white socks), distinctive features, little detail,
and solid colors or backgrounds require less attention, effort, and
visual analysis than do activities that involve choosing among
items that have low contrast (e.g., light beige and white socks),
are in unusual positions, are embedded within crowded or
distracting visual backgrounds, or are partially obscured.
Changes in the familiarity, number of items, and degree of detail
can place greater demands on visual processing. In addition,
verbal mediation, including repeating a list of step-by step
instructions during a functional activity such as dressing,
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capitalizes on strengths in verbal abilities and can be effective in
facilitating functional performance (Sunderland, Walker, &
Walker, 2006).
Motor Planning
Motor planning, or praxis, is the ability to execute learned and
purposeful activities. Apraxia is defined as a disorder of skilled
movement that cannot be adequately explained by primary motor
or sensory impairments, visual spatial problems, language
comprehension difficulties, or cognitive problems alone (Heilman
& Rothi, 2003). Damage to the association areas of the brain
(affecting the cognitive aspects of motor control) is thought to
cause apraxia (Kertesz, 1982). Apraxia may be seen after strokes
in either hemisphere, although it is more commonly seen in
clients who have sustained a left-hemisphere lesion. Aphasia is
often associated with apraxia, since the left hemisphere is also
dominant for language (Heilman & Rothi, 2003). People with
apraxia can improve performance of skilled movement over time
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(Basso, Burgio, Paulin, & Prandoni, 2000); however, they
frequently continue to have significant functional limitations in
both the learning of new motor tasks, such as one-handed shoe
tying (Poole, 1998), and in the performance of motor acts to
verbal command or demonstration Roy (1978) identifies two
major subsystems in apraxia: the conceptual and the production
subsystems. The symptoms of apraxia may reflect disorders in
one or both of these subsystems. The production aspect of motor
planning, traditionally called ideomotor apraxia, involves
generating the action plan, sequencing and organizing the
appropriate elements, and carrying out the plan (e.g., reaching
for a glass of water to take a drink). The greatest difficulty is
observed when the client is asked to pretend use of a tool or
object or to perform limb gestures. Some improvement may be
seen when the client is asked to imitate the motion or perform
the motion with the actual object, but the movement is still
imprecise. These clients know what they want to do, but actions
are carried out in an awkward, inefficient, or clumsy manner.
Errors of preservation, sequencing, or omissions may be
observed. The conceptual aspect of motor planning (Roy, 1978)
includes knowledge about the functional properties of an object,
the object action, and the sequence of action. Conceptual errors,
traditionally called ideational apraxia, involve object function,
action knowledge, and knowledge of sequence. Clients might be
able to accurately identify and match objects, but inappropriate
use of objects is frequently observed. For example, the client
might try to brush his or her hair with a toothbrush. Although
object recognition may be intact, the person might be unable to
associate the object with its correct action plan. Dressing apraxia
and constructional apraxia are additional subtypes of apraxia,
previously described in the section on visual processing.
Traditional labels of apraxia are narrow in scope and do not
account for the wide range of skills that underlie motor planning
and constructional abilities.
Evaluation
The classic definition of apraxia includes motor-planning
problems that cannot be accounted for by weakness, increased
tone, incoordination, sensory loss, or other cognitive-perceptual
impairments. The clinician is urged to analyze underlying
reasons for difficulties in performance rather than attempting to
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classify clients within traditional categories. In clinical practice,
most clients have associated difficulties that contribute to
difficulty in motor planning. Information on the client’s language
skills should be obtained from the speech-language pathologist
or be screened for by testing for “yes” or “no” comprehension and
ability to follow one-step commands, since apraxia and aphasia
often coexist. In evaluating apraxia, the clinician typically
observes the client’s performance of different types of movements,
noting the method of evocation (e.g., command, imitation, or
object use) and type of errors made (Haaland, 1993).
Assessments for apraxia are listed in Table 57.1. An
observational method for assessing apraxia in ADL activities,
adapted from the Arnadottir OT Neurobehavioral Evaluation (A-
ONE), has been validated for people with stroke (van Heugten et
al., 2000). A dynamic assessment approach attempts to identify
the activity conditions under which the limb apraxia symptoms
emerge, the client’s response to cueing, and the client’s
awareness of his or her activity performance (Toglia, 1998).
Intervention
Interventions to overcome motor-planning deficits may emphasize
either the production aspect or the conceptual aspect of motor
planning (Roy, 1985). Techniques that address the orientation of
an object or limb in space or the timing, sequence, and
organization of the motor elements aim to enhance the
production aspect of motor planning. For example, the
practitioner might provide physical con- tact (i.e., hand-over-
hand assistance or light touch) to limit inappropriate or
extraneous movements while simultaneously using guiding
methods to facilitate a smooth motor pattern or to guide the
manipulation of objects. Through repeated practice in different
tasks, the client begins to learn the movement patterns that feel
“right,” and the practitioner gradually withdraws assistance.
Deep proprioceptive input and contact have an inhibitory effect
on normal people, whereas light touch tends to have a more
facilitatory effect (Farber, 1993). Familiar tasks that are
performed in context are easier for people with motor-planning
disorders because the context provide cues that facilitate the
desired action (Ferguson & Trombly, 1997). Interventions can be
graded by gradually introducing activities and environments that
have less stability and predictability, such as negotiating around
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obstacles in a crowded store. Intervention addressing the
conceptual aspect of motor planning focuses on facilitating the
client’s understanding of how an object is used or how a gesture
is performed (Helm-Estabrooks, 1982; Pilgrim & Humphreys,
1994; Smania, Girardi, Domenicali, Lora, & Aglioti, 2000).
STRATEGY TRAINING
Clients may be taught to use verbal, visual, or tactile cues to
enhance movement. For example, before performing an activity,
the client might mentally practice or imagine the task
performance; or the client might imagine how an object should
look in his or her hand before picking it up. Incorrect patterns of
movement, such as holding an object the wrong way, can also be
visualized, with an emphasis on having the client mentally
practice correcting the movement. Talking a client through action
sequences or use of step-by-step written lists or illustrations can
be useful in facilitating functional performance in tasks such as
drinking from a cup (Butler, 1999). The person can be taught to
verbally rehearse an action sequence or associate the movement
with a rhyme, rhythm, or musical tune with a gradual fading of
the verbalization. Self-monitoring strategies can be used to teach
a client to monitor unnecessary cocontraction, incomplete
actions, or difficulty in switching direction of movements.
Preliminary studies indicate that strategy training is effective in
improving everyday function (Donkervoort, Dekker, Stehmann-
Saris, & Deelman, 2001; Geusgens et al., 2006). For example, in
a randomized study design, changes on nontrained ADL activities
were greater in a group of people with stroke who had received
strategy training as compared with those receiving usual
occupational therapy. This suggests that the strategies
generalized to everyday activities (Geusgens et al., 2006).
Memory
Memory gives us the ability to draw on past experiences and
learn new information (Toglia, 1993a). This provides us with a
sense of continuity in the environment and frees us from
dependency in here-and-now situations. Memory is
conceptualized as a multistep process involving encoding (i.e.,
input of information), storage (i.e., hold- ing information), and
retrieval (i.e., getting information) (Levy, 2005b). There are
different types of memory. Working memory is the temporary
storage of information while one is working with it or attending to
it. It includes the ability to recall information immediately after
exposure. It allows one to focus conscious attention and keep
track of information as one is performing an activity. Declarative
memory is one aspect of long-term memory and includes
conscious memory for events, knowledge, or facts. Procedural
(nondeclarative) memory involves the ability to remember how to
perform an activity or procedure without conscious awareness.
Prospective memory involves the ability to remember intentions
or activities that will be required in the future (Levy, 2005b).
Evaluation
It is important to distinguish whether everyday memory problems
are due to failures to recall past events or conversations or
failures in carrying out future activities (e.g., prospective
memory). A comprehensive evaluation of memory, whether static
or dynamic, must address the different types of memory and
methods of retrieval (Table 57.1). Assessments must consider
factors such as the modality in which the information is
presented (auditory or visual), the type of instructions (general or
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specific), the amount of stimuli presented, the familiarity and
meaningfulness of the information, the presence of contextual
cues during recall phases, the type of information to be remem-
bered (factual or skill related), and the length of retention.
Dynamic assessment of memory, such as Toglia’s (1993b)
Contextual Memory Test, evaluates awareness of memory
capabilities and use of strategies.
Intervention
Memory impairments can be closely related to other cognitive
impairments, particularly attention. Some investigators have
suggested that an indirect approach that addresses other
cognitive skills, such as attention or organization, rather than
memory, may be effective. For example, Sohlberg and Mateer
(1989a) reported improvement in memory function after
attentional training. Interventions for memory impairments
include memory strategy training, external aids and devices, and
adaptations, as well as techniques of errorless learning,
vanishing cues, and spaced retrieval that were discussed earlier
in this chapter.
STRATEGY TRAINING
Training of internal memory strategies is most appropriate for
people with mild memory deficits or those in whom other areas of
cognition are intact (Cicerone et al., 2000, 2005). The client
practices one or two targeted memory strategies in a variety of
different tasks, such as remembering telephone numbers, news
headlines, a sequence of errands, items that need to be bought in
a store, or instructions to an activity. During practice on different
memory tasks, a variety of awareness training techniques may
also be used. Memory strategies may be directed primarily at
encoding operations (i.e.,getting information in) or the retrieval
phase of memory (i.e., getting information out). Encoding
strategies include the following:
Chunking or grouping similar items
The story method, or linking a series of facts or events into
a story
Rehearsal, or repeating information over and over silently
Rhymes, or recalling a fact by changing the fact into a
rhyme
Visual imagery
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Retrieval strategies include the following:
Alphabetical searching, or going through the alphabet to
find the first letter of a forgotten item
Retracing one’s steps to find a missing object or to recall an
event
Thinking of associated information to cue the recall of a new
fact or event
Self-generating words, concepts, or items to improve
learning and memory (Chiaravalloti & DeLuca., 2002)
MEMORY EXTERNAL STRATEGIES AND AIDS. External aids
such as notebooks, tape recorders and computers store
information that the person might have difficulty remembering.
Other aids such as pagers or alarm signals serve to remind a
person to perform an action (prospective memory) (Toglia, 1993a).
The success of an intervention program that utilized a
combination of external aids and strategies with awareness
training to improve prospective memory was recently described
by Fleming, Shum, Strong, and Lightbody (2005). External
memory aids include the following: timers, tape recorders,
devices with preprogrammed alarms or alarm messages,
electronic devices such as pagers, mobile phones, palm pilots,
and cell phones, computers, pill box organizers, lists, daily plan-
ners, and notebooks (Figure 57.3). Case studies have
documented the effectiveness of external aids (McKerracher,
Powell, & Oyebode, 2005; Wade & Troy, 2001; Wilson, Emslie,
Quirk, & Evans, 2001). Intervention is most effective when the
client is motivated, involved in identifying the memory problem,
and fairly independent in daily function (Cicerone et al., 2000,
2005). Evidence obtained from case studies supports the use of
memory notebooks and other external aids in reducing everyday
memory failures for people with moderate to severe memory
impairments (Cicerone et al., 2000, 2005, McKerracher et al.,
2005; Wade & Troy, 2001; Wilson et al., 2001). However, the
successful use of an external memory aid may require extensive
training. The client may need to practice initiating and using the
aid in a variety of different situations. The use of external aids
might need to be graded. In the initial stages, the client might be
expected to use the aid only when it is initiated by another
person. Gradually, the client might be trained to initiate the use
of the aid independently. Errorless learning, spaced retrieval, and
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other task-specific training methods that capitalize on procedural
memory, may be used in training clients with moderate or severe
memory impairments to use an external aid. The most commonly
used external memory strategy is the memory notebook. The
memory notebook needs to be designed with the person’s needs
and lifestyle in mind (McKerracher et al., 2005). Sample sections
in a memory notebook are as follows: personal facts, names of
people to remember, calendar and schedule, things to do
important events (daily, within the next week), daily log of
important events, conversations, summary of readings (articles,
newspaper), medication schedule, and directions to frequently
traveled places. Initially, the notebook should begin with one or
two sections and gradually increase. Memory notebook training
needs to take place in the context of a variety of everyday
activities. Therapy sessions should include role-playing and
practice in use of the notebook. In addition, the client may be
asked questions that involve reviewing and rereading the memory
notebook. Specific memory notebook training protocols have been
described in the literature (Donaghy & Williams 1998; Sohlberg &
Mateer, 2001).
Evaluation
Most standardized cognitive assessments are structured and do
not adequately examine the area of executive functions (Sohlberg
& Mateer, 2001) (see Table 57.1). Several assessments for
executive functions have recently been developed (Bamdad, Ryan,
& Warden, 2003; Birnboim & Miller, 2004; Wilson, Alderman,
Burgess, Emslie, & Evans, 1996). Although these assessments
appear more “ecologically valid” (i.e., able to predict behavior in
everyday situations) than previous assessments were, further
research data on the reliability and validity of these assessment
tools are needed.
INTERVENTION
STRATEGY TRAINING
Strategies that maximize executive functioning can be practiced
in a variety of unstructured tasks that require initiation,
planning, organization, and decision making, such as organizing
medications according to a schedule, planning an overnight trip
and packing a suitcase, obtaining and organizing a list of local
business phone numbers, and organizing tools Verbal mediation
has been reported to be an effective strategy in improving
executive function and self-regulation deficits. For example,
Cicerone and Wood (1987) reported the successful use of a self-
instructional procedure in a client with impaired planning ability
and poor self-control secondary to brain injury. Intervention
involved requiring the client to verbalize a plan of action before
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and during execution of a task. Gradually, the client was
instructed to whisper rather than talk aloud. Generalization to
real-life situations was observed after an extended period of time
that included training in self-monitoring. Training in problem
solving strategies involves teaching the person to break down
complex activities into smaller, more manageable steps.
Strategies may also aim to help the person to maintain the focus
of goals and intentions (Katz & Hartman-Maeir, 2005). An
evidenced based review (Cicerone et al., 2000, 2005) concluded
that there is evidence to support the use of formal problem-
solving training with application to everyday activities. The
authors recommended such training as a practice guideline for
people with stroke or brain injury during postacute
rehabilitation. The intervention goal is to replace an impulsive,
disorganized approach with a systematic and controlled approach
to planning activities, maintaining goal intentions, and solving
problems. The steps of the problem-solving process are reinforced
with use of self-questioning techniques. For example, self-
questioning cue cards with the following types of questions can
be used during problem-solving tasks: What do I need to do? Do I
need more information? What do I have to do next? Have I
identified all the critical information? Do I understand the
problem? What are all the possible solutions? Did I choose the
best one? Broad checklists or task guidance systems are
commonly used to assist the client in initiating, planning, and
carrying out an activity systematically. Checklists may be specific
to a particular activity (e.g., following steps to operate a computer
program), or they may be designed broadly so that they can be
used in a variety of similar activities (e.g., a checklist for food
preparation or cooking activities). Interventions should
incorporate practice in identifying the situations or activities in
which use of a checklist could be helpful. The client may be given
the opportunity to practice the same activity with and without
the use of a checklist to enhance awareness. Initially, the goal
might be to have a client follow a checklist established by the
practitioner or significant other. Eventually, the client might be
given checklists with missing steps and be asked to review the
lists to identify the missing components. Finally, the client might
be required to create a checklist independently. Burke, Zencius,
Wesolowskis, and Doubleday (1991) describe four cases of
individuals with executive dysfunction for whom checklists were
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successfully used to improve the ability to carry out routine
vocational tasks. Decreased initiation, one of the hallmark
features executive dysfunction, can significantly interfere with
the ability to use and apply a learned strategy. For example, a
person with deficits in executive functions might use a strategy
effectively when cued but not use the strategy spontaneously
because of a failure to initiate its use. External cues such as
alarm signals can be used to prompt the client to initiate a task,
switch to a different task component, or use a particular strategy
within an activity (Evans, Emslie, & Wilson, 1998; Manly,
Hawkins, Evans, Wodlt, & Robertson, 2002).
GROUP INTERVENTIONS
Cognitive rehabilitation principles and strategies can be
incorporated within group programs and combined with
psychosocial or psychoeducational interventions. The group
format can be used to target specific cognitive skills, or it can be
used to teach compensatory strategies (Revheim & Marcopulos,
2006; Schwarztberg, 1999; Stuss et al., 2007). Group activities
can emphasize interpersonal skills within cooperative tasks, such
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as planning a bake sale or publishing a newsletter, or role-
playing scenarios involving interviews, conflicts, or on-the-spot
problem solving. Strategies that include monitoring the tendency
to respond impulsively, become stuck in one viewpoint, or
wander off task can be practiced within social contexts (Toglia,
2005). Group programs that center on teaching self-monitoring
techniques and strategies for paying attention, remembering,
organization, or problem solving can be applied to a wide
spectrum of clients. Activities such as remembering names and
facts about group members, recalling directions for operating a
new electronic device, or creating a checklist for a complex task
can provide opportunity to practice different strategies and share
experiences within a group context. Group members can be
encouraged to reflect on performance and identify strategies that
would be useful in their everyday activities. Group interventions
that simultaneously address subtle cognitive difficulties and
emotional issues have demonstrated value in improving self
awareness, self-efficacy, coping skills, psychosocial skills, and
perceived daily functioning (Harrison et al., 2005; Rath, Smon,
Langenbahn, Sherr, & Diller, 2003; Toglia, 2005).
SUMMARY
Recently, there has been a move away from intervention
programs that focus exclusively on remediation of cognitive
impairments. There is increasing evidence that supports the use
of comprehensive and holistic cognitive rehabilitation programs
that address a combination of cognitive, emotional, functional,
and social participation skills in people with brain injury (Cappa
et al., 2005; Cicerone et al., 2005, Cicerone, Mott, Azulay, &
Friel, 2004; Sarajuuri et al., 2005; Tiersky et al., 2005). The need
to blend cognitive interventions with those that address
interpersonal and real-world functioning has been emphasized in
recent literature; however, the outcome of cognitive rehabilitation
is most commonly measured at the impairment level. As
occupational therapists return to more community focused
intervention, we need to widen our perspective on the influence
that cognitive perceptual impairments have on our clients’ ability
to engage in the occupations they need or want to do within the
contexts of their lives. We need to explore the effect of cognitive
rehabilitation on occu- pational engagement and social
participation, for even subtle cognitive impairments can decrease
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satisfaction, participation, and quality of life, preventing our
clients from leading enriching lives (McDowd, Filion, Pohl,
Richards, & Stiers, 2003). The outcome or benefit of cognitive
rehabilitation needs to be examined broadly across different
populations, including effects on changing existing habits;
routines or increasing productive activity patterns; increasing the
frequency and quality of social participation; decreasing caregiver
assistance, stress, or burden; improving subjectivwell-being,
including self-efficacy, self-esteem, satisfaction and quality of life;
and preventing functional decline.
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CHAPTER 14
MOTOR NEURON DISEASE
AMYOTROPHIC LATERAL SCLEROSIS
TYPICAL COURSE
The onset of the disease varies from one individual to another.
Some people experience a weakness in the arms with difficulty
lifting or doing fine motor tasks or in the legs with difficulty
walking or in the muscles that control speech and swallowing.
Some experience generalizedmuscle fatigue. If the weakening
begins in the hand, it will progress through the affected limb
before becoming more generalized. Problems with speech or
swallowing occur when motor neurons die in the brain stem. The
disease progresses and eventually affects the person’s ability to
walk and continue performing ADL. The disease affects only the
motor pathways; therefore, eye movement, bowel and bladder
functions, cognition, personality, and skin sensation remain
intact. Respiratory weakness ultimately is affected as the
muscles deteriorate, and death usually follows unless the
individual is put on a ventilator.
POTENTIAL SYMPTOMS
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Damage to lower motor neurons (in the spinal cord) leading
to flaccid paralysis, decreased muscle tone, and decreased
reflexes
Damage to upper motor neurons (in the brain) and to the
corticospinal tract leading to spasticity and hyper- reflexia
(exaggerated reflexes)
Muscle weakness
Muscle atrophy (distal to proximal)—a symptom that is
unique to ALS
Fatigue
Stumbling and falling due to lower extremity weakness
Fasciculation (muscle twitching)
Decreased ability to regulate body temperature
Loss of emotional control/depression
Dysphagia (difficulty swallowing)
Dysarthria (difficulty speaking) due to impaired cranial
nerves controlling speech
Impaired respiration due to muscle weakness
Sialorrhea (excess drooling)
Night cramps
Weight loss
Loss of endurance
Loss of dexterity
MEDICAL MANAGEMENT
No specific assessment is used to diagnose ALS. Magnetic
resonance imaging (MRI), electromyography, blood tests, and/or
nerve conduction velocity are done to eliminate other diseases as
possibilities. Currently, no known cure or treatment to reverse
ALS exists. In 1995, the Food and Drug Administration approved
Rilozule, the first drug to result in prolonging survival by several
months. The drug is believed to work by decreasing damage due
to the release of glutamate. The drug may extend the length of
time before a person needs ventilation support. Respiratory
therapists, speech pathologists, physical therapists, occupational
therapists, psychologists, and social workers typically work
together to sustain the client’s quality of life and to support the
family. Compensatory strategies using adaptive equipment and
energy conservation are common foci of treatment for an
individual with ALS.
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PROGNOSIS
Most people with ALS die secondary to respiratory failure
People who go on a ventilator generally live longer than
people who do not
90% of people with ALS live 3–5 years after the onset of the
disease
10% of people with ALS survive for 10 or more years after
the onset
PRECAUTIONS
Pneumonia and pulmonary emboli
Ventilators: intermittent positive pressure ventilation (IPPV)
or bilevel positive airway pressure (BiPap)
Inability to cough to clear normal amount of mucus from
airway
Pressure sores due to decreased mobility
Swallowing problems could lead to choking
Posture and balance could lead to falls
Emotional lability (outbursts of laughing or crying)
Difficulty maintaining weight
Shoulder subluxation
Joint contractures
CAREGIVER CONCERNS
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People who develop ALS experience a sudden change in life roles
that can be overwhelming. Because of the rapid progression of
ALS, it can be difficult for caregivers to adapt to a loved one’s
inability to continue life roles. Discontinuation of work after the
diagnosis of ALS is common and can lead to financial strain. The
sudden decrease in ADL/IADL capabilities can be difficult for the
family. Caring for a family member with ALS can be physically
and psychologically demanding and draining. It is important to
address the issue of coping not only with the client but also with
the family members. Because so much is unknown about ALS,
caregivers often have many unanswered questions. Caregiver
support groups can be beneficial in providing the family with
necessary support and information.
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CHAPTER 15
BELLY'S PALSY
Aetiology
Uncertain, but may be associated with viral infections, e.g.
herpes simplex and varicella- zoster; epidemics of Bell’s palsy
occur sporadically.
Symptoms
Pain of variable intensity over the ipsilateral mastoid precedes
weakness, which develops over a 48hour period. Impairment of
taste, hyperacusis and salivation depend on the extent of
inflammation and will be lost in more severe cases. Lacrimation
is seldom affected.
Prognosis
Most patients (70%) recover in 4–8 weeks without treatment. In
the remainder, residual facial asymmetry may require corrective
surgery. Incomplete paralysis indicates a good prognosis. In
patients with complete paralysis, electrical absence of
denervation on electromyography is an optimistic sign.
Occasionally aberrant reinnervation occurs movement of the
angle of the mouth on closing the eyes (jaw winking) or
lacrimation when facial muscles contract (crocodile tears). On
attempting to close the eyes and show the teeth, the one eye
does not close and the eyeball rotates upwards and outwards
Bell’s phenomenon (normal eyeball movement on eye closure).
132
Diagnosis
Based on typical presentation and exclusion of middle ear
disease, diabetes, sarcoidosis and Lyme disease.
133
CHAPTER 16
MOVEMENT DISORDERS
Cerebellar Dysfunction
Three major phylogenetic subdivisions of the cerebellum are
recognised.
1. The anterior lobe (paleocerebellum)
2. The posterior lobe (neocerebellum)
3. The flocculonodular lobe (archicerebellum)
CEREBELLAR DYSFUNCTION
CLINICAL PRESENTATION, ANATOMICAL CONCEPTS AND
DIAGNOSTIC APPROACH
180 Anatomy
The cerebellum lies in the posterior fossa, posterior to the brain
stem, separated from the cerebrum above by the tentorium
cerebelli. The cerebellum consists of two laterally placed
hemispheres and the midline structure the vermis. Receives
afferent fibres from (spinocerebellar pathways) in the spinal cord.
135
Titubation
Titubation is a rhythmic ‘nodding’ tremor of the head from side to
side or to and fro, usually associated with distal limb tremor. It
appears to be of little localising value.
Head tilt
Abnormal head tilt suggests a lesion of the anterior vermis. Note
that a IV (trochlear) cranial nerve palsy and tonsillar herniation
also produce this abnormal posture.
Involuntary movements
Myoclonic jerks and choreiform involuntary movements occur
with extensive cerebellar disease involving the deep nuclei.
NOTE: Cerebellar lesions may cause symptoms and signs
relating to
obstructive hydrocephalus
cranial nerve involvement
brain stem involvement.
(Extensor spasms from brain stem damage may be wrongly
described as ‘cerebellar fits’.)
Nystagmus
Nystagmus is defined as an involuntary ‘to and fro’ movement of
the eyes in a horizontal, vertical, rotatory or mixed direction. The
presence and characteristics of such movements help localise to
the site of neurological disease.Nystagmus may be pendular –
equal velocity and amplitude in all directions, or jerk – with a fast
phase (specifying the direction) and a slow phase. The normal
maintenance of ocular posture and alignment of the eyes with the
environment
Nystagmus may result from:
retinal disease
labyrinthine disease, or
disorders affecting the cerebellum or a substantial portion of
the brain stem.
137
VESTIBULAR NYSTAGMUS
Nystagmus arises from:
natural stimulation of the vestibular apparatus
rotational or linear acceleration.
artificially removing or increasing the stimulus from one
labyrinth (e.g. caloric testing).
damage to vestibular apparatus or the vestibular nerve.
Physiological
1. Rotational acceleration produces nystagmus in the plane of
rotation. Creates an imbalance between each side resulting
in a slow drift of the eyes towards the damaged side (or side
with the reduction in stimulus) followed by a fast
compensatory movement to the opposite side. Slow phase in
a direction tending to maintain the visual image. Fast phase
in the opposite direction. Slow Fast Slow Fast
2. Caloric testing sets up convection currents in the lateral
semicircular canal producing a horizontal nystagmus (see
page 65).
Pathological
Damage to labyrinth or vestibular nerve. Slow Fast Slow phase to
side of lesion. Quick or fast phase to normal side. Rotatory
component often present.Turning eyes away from the side of the
lesion increases amplitude but does not change direction of
nystagmus. In severe cases, the nystagmus is 3rd degree and
gradually settles to 1st degree with recovery. Enhanced by loss of
ocular fixation. Vertigo accompanies nystagmus. After a delay of
several seconds, nystagmus develops often with a rotatory
component. With repeated testing, the nystagmus fatigues. To
elicit, suddenly reposition the patient: Often associated with
tinnitus and hearing loss. Vertigo and nystagmus settle
simultaneously. Occurs in acute labyrinthine disease Menière’s
disease, vestibular neuronitis, vascular disease.
POSITIONAL
nystagmus: this may occur in labyrinthine disease in association
with INTRACRANIAL TUMOURS
AETIOLOGY/INCIDENCE
AETIOLOGY (cont’d)
138
a. Inactivation of expression of tumour suppressor genes (e.g.
mutation of the p53 gene with loss of heterozygosity on the
17p chromosome in many patients with low grade
astrocytoma).
b. Over expression of genes controlling growth factor (e.g.
amplification of EGFR in primary glioblastoma).
Clearly defined inherited factors play a minor role. Only 5% of
patients have a family history of brain tumour and with the
exception of tuberous sclerosis (related to the formation of
subependymal astrocytomas) and neurofibromatosis (linked to an
increased incidence of schwannoma, optic nerve glioma and
meningioma) do not fall into an obvious autosomal recessive or
dominant pattern. Others include von Hippel-Lindau disease,
Cowden’s disease and Li-Fraumeni syndrome.
Cranial irradiation: long term follow-up of patients undergoing
whole head irradiation for treatment of tinea capitis and
childhood leukemia shows an increased incidence of both benign
and malignant tumours e.g. astrocytoma, meningioma.
Immunosuppression: increased incidence of lymphoma.
INCIDENCE
The table below shows the approximate incidence of intracranial
tumours extracted from large series.
Adults Children
Glioblastoma 15% Medulloblastoma/PNET 16% Low grade glioma
5% Low grade glioma 33% Meningioma 25% Malignant glioma
14% Pituitary adenoma 25% Ependymoma 10%Primary CNS
Lymphoma 4% Craniopharyngioma 6% Peripheral Nerve Sheath
Tumour 8% Germ cell tumours 2.5% (schwannoma) Meningioma
2.5% Others 18% Others 16% Adapted from Louis et al. Adapted
from Rickert & Paulus Child’s WHO Classification of Tumours
Nervous System Symptoms tend to develop insidiously, gradually
progressing over a few weeks or years, depending on the degree of
malignancy (cf. acute onset of a cerebrovascular accident
followed by a gradual improvement if the patient survives).
Occasionally tumours present acutely due to haemorrhage or the
development of hydrocephalus.
HYPOTHALAMUS/PITUITARY
Endocrine dysfunction.
Right/left confusion
Finger agnosia dominant
Acalculia hemisphere
Agraphia
PARIETAL LOBE
Disturbed sensation
localisation of touch
two point discrimination
passive movement
astereognosis
sensory inattention
Visual field defect
lower homonymous quadrantanopia
140
N.B. Intrinsic brain stem tumours in contrast to extrinsic
tumours are more likely to produce long tract (motor and
sensory) signs early in the course of the disease.
CEREBELLUM
Ataxic gait
Intention tremor
Dysmetria
Dysarthria
Nystagmus
Infratentorial
MIDBRAIN/BRAIN STEM
Cranial nerve lesions III–XII
Long tract signs – motor and sensory
Deterioration of conscious level
Tremor (red nucleus)
Impaired eye movements
Pupillary abnormalities
Vomiting, hiccough
MASS EFFECT
midline shift.
ventricular compression.
hydrocephalus (secondary to 3rd ventricular or posterior
fossa lesion).
obliteration of basal cisterns
CT scanning Note: SITE e.g. frontal, occipital
extrinsic: outwith brain substance, e.g. meningioma
intrinsic: within brain parenchyma, e.g. astrocytoma.
141
useful in demonstrating the vertical extent of a tumour and
its relationship with other structures, especially when
intraventricular or arising from the pituitary fossa or skull
base.
HIGH DEFINITION SCANS (1 mm slice width) useful in the
detection of pituitary, orbital and posterior fossa tumours.
STEROID THERAPY
Steroids dramatically reduce oedema surrounding intracranial
tumours, but do not affect tumour growth. A loading dose of 12
mg i.v. dexamethasone followed by 4 mg q.i.d. orally or by
injection often reverses progressive clinical deterioration within a
few hours. After several days treatment, gradual dose reduction
minimises the risk of unwanted side effects. Sellar/parasellar
tumours occasionally present with steroid insufficiency. In these
patients, steroid cover is an essential prerequisite of any
anaesthetic or operative procedure.
OPERATIVE MANAGEMENT
Most patients with intracranial tumours require one or more of
the following approaches:
Craniotomy: flap of bone cut and reflected.
If necessary, combined with image guidance to aid positioning
the flap and to give accurate lesion localisation
142
Transphenoidal route: through the sphenoid sinus to the
pituitary fossa
Burr hole: for stereotactic or hand-held, ultrasound
guided biopsy
Transoral route: removal of the arch of the atlas, odontoid peg
and clivus provides access to the anterior aspect of the brain
stem and upper cervical cord. Rarely required for anteriorly
situated tumours, e.g. neurofibromas, chordoma.
Craniectomy: burr hole followed by removal of surrounding
bone to extend the exposure routinely used to approach the
posterior fossa
RADIOTHERAPY
Treatment of intracranial tumours with radiotherapy utilises one
of the following:
megavoltage X-rays (by far the most common method)
electron beam from a linear accelerator (which can also
produce megavoltage X-rays)
accelerated particles from a cyclotron, e.g. nuclei of helium,
protons (awaits full evaluation)
γ rays from cobalt 60.
In contrast to older methods, these modern techniques produce
greater tissue penetration and avoid radiation damage to the skin
surface. The effect of radiotherapy depends on the total dose
144
usually up to 60 Gy, and the treatment duration. This must be
balanced against the risk to normal structures. Treatment aims
to provide the highest possible dose to a specified region whilst
minimising irradiation to adjacent normal brain. Various
methods have been developed to achieve this
Conformal therapy where standard radiotherapy is
administered, but the beams are shaped by the use of
variable collimators or blocks which conform with the shape
of the tumour, thereby eliminating normal brain.
Stereotactic radiosurgery (SRS) where multiple converging
beams from a linear accelerator or from multiple cobalt60
sources are focused on a selected target in a single
treatment. Stereotactic radiotherapy (SRT) uses the same
localisation method but with fractionated treatment as used
in conventional radiotherapy (see page 385).
Interstitial techniques where the tumour is treated from
within (brachytherapy) by the implantation of multiple
radioactive seeds, e.g. iodine125.
Beam intensity modulated radiotherapy (IMRT) uses non-
uniform beams of varying intensity (in contrast to the
conventional uniform dose intensity) to complex tumour
volumes. This helps protect surrounding structures, yet
allows a higher dose. Proton therapy is available in only a
few centres. It allows the delivery of high doses of radiation
to very localised regions adjacent to vital structures such as
the skull base.
Radiotherapy is of particular value in the management of
malignant tumours malignant astrocytoma, metastasis,
medulloblastoma and germinoma, but also plays an important
part in the management of some benign tumours – pituitary
adenoma, craniopharyngioma. With some tumours that seed
throughout the CSF pathways, e.g. medulloblastoma, whole
neural axis irradiation minimises the risk of a distant recurrence.
Complications of radiotherapy: following treatment, deterioration
in a patient’s condition may occur for a variety of reasons:
Increased oedema – during treatment – reversible.
Demyelination – after weeks, months – usually reversible.
Radionecrosis – in usually 1–2 years (range 6 months–10
years) – irreversible.
Cognitive impairment – whole brain irradiation causes
dementia, ataxia and incontinence in over 10% at one year.
145
Radiotherapy should be avoided in children under 3 years of
age.
Radiation induced tumours e.g. meningioma, may result
many years after the treatment.
Oedema, demyelination and radionecrosis may involve the spinal
cord after irradiation of spinal tumours. Other harmful effects
include hair loss, skin reactions and endocrine reaction.
CHEMOTHERAPY
Chemotherapeutic agents have been used for many years in the
management of malignant brain tumours, but their benefits
remain limited. Historically drugs most commonly used include
nitrosoureas (e.g. BCNU, CCNU), procarbazine, vincristine and
methotrexate (for lymphoma). Temozolomide, an oral alkylating
agent with excellent blood brain barrier penetration and modest
toxicity is established as an alternative treatment for patients
with recurrent high grade gliomas. It has also been shown to
improve survival for patients with newly diagnosed glioblastoma
when given concomitantly with radiotherapy. A combination of
maximal safe surgery followed by combined chemoradiotherapy is
now the standard of care for good performance patients with
glioblastoma. Patients with methylation of the MGMT gene in the
tumour appear particularly to benefit. Carmustine impregnated
wafers (Gliadel) may also be considered both as a primary
treatment or for tumour recurrence (see below). Patients with
anaplastic oligodendrogliomas and oligoastrocytoma with loss of
heterozygosity on chromosomes 1p and 19q have a good
prognosis and respond well to both radiation and to alkylating
agent based chemotherapy (nitrosoureas, Temozolomide).
Chemotherapy may be used either at initial diagnosis or at
relapse in these patients. Other tumours where chemotherapy
plays an important role include medullo-blastomas, primary CNS
lymphomas and germ cell tumours. Traditionally, chemotherapy
has had a lesser role in low grade glial tumours but current
studies are examining its use in both astrocytomas and
oligodendrogliomas as an alternative to radiation in newly
diagnosed patients. Problems of drug administration
Toxicity: The ideal cytotoxic drug selectively kills
146
CHAPTER 17
HYDROCEPHALUS LOCALISED NEUROLOGICAL DISEASE
AND ITS MANAGEMENT A. INTRACRANIAL
DEFINITION
CLASSIFICATION
‘Obstructive’ hydrocephalus – obstruction of CSF flow within the
ventricular system. ‘Communicating’ hydrocephalus obstruction
to CSF flow outwith the ventricular system i.e. ventricular CSF
‘communicates’ with the subarachnoid space.
CAUSES OF HYDROCEPHALUS
Obstructive Communicating Acquired – Acquired aqueduct
stenosis Thickening of the leptomeninges (adhesions following
infection and/or involvement of the arachnoid or haemorrhage)
granulations
Supratentorial masses causing infection (pyogenic, TB,
fungal) tentorial herniation subarachnoid haemorrhage
Intraventricular haematoma spontaneous
Tumours ventricular, e.g. traumacolloid cyst postoperative
pineal region carcinomatous meningitis
posterior fossa Increased CSF viscosity, e.g.
Abscesses/granuloma high protein content
147
Arachnoid cysts Excessive CSF production choroid plexus
papilloma (rare)
Congenital – Aqueduct stenosis or forking
Dandy-Walker syndrome (atresia of foramina of Magendie
and Luschka)
Chiari malformation
PATHOLOGICAL EFFECTS
In the infant, prior to suture fusion, head expansion and massive
ventricular dilatation may occur, often leaving only a thin rim of
cerebral ‘mantle’. Untreated, death may result, but in many cases
the hydrocephalus ‘arrests’; although the ventricles remain
dilated, intracranial pressure (ICP) returns to normal and CSF
absorption appears to balance production. When hydrocephalus
arrests, normal developmental patterns resume, although pre-
existing mental or physical damage may leave a permanent
handicap. In these patients, the rapid return of further pressure
symptoms following a minor injury or infection suggests that the
CSF
INVESTIGATIONS
Skull X-ray
Note: – skull size and suture width.
Evidence of chronic raised pressure – posterior clinoid
erosion, ‘copper beating’.
associated defects – platybasia, basilar invagination.
CT scan
The pattern of ventricular enlargement helps determine the
cause, i.e. Periventricular lucency (if present normal 4th –
suggests suggests raised CS lateral ventricle aqueduct pressure.
stenosis. (Wide sulci suggests 3rd ventricular deviated or –
suggests a ventricular
Dilatation
MANAGEMENT
Acute ventricular drainage or deterioration ventriculo-peritoneal
(VP) shunt or 3rd ventriculostomy (if tri-ventricular – obstructive
hydrocephalus) lumbar puncture if communicating
148
hydrocephalus, e.g. following subarachnoid haemorrhage.
Gradual VP shunt (lumboperitoneal shunts are occasionally used
for deterioration communicating hydrocephalus) or 3rd
ventriculostomy. removal of a mass lesion if present – this may
obviate the need for a shunt. ‘Arrested hydrocephalus’
symptomless ventricular dilatation requires no treatment, but
regular developmental or psychometric assessment ensures no ill
effects develop from this potentially unstable state.
Shunt techniques
A reservoir permits CSF aspiration for analysis.
A valve is incorporated in the system, with either fixed opening
pressure e.g. Heyer-Schulte, Hakim variable opening pressure
(flow regulated) e.g. Orbis sigma, Delta programmable e.g. Medos,
Sophy. Valve opening pressures range from 5–150 mmH2O
[Lumboperitoneal shunt catheter inserted into the lumbar theca
either directly at open operation or percutaneously through a
Tuohy needle. The distal end is sited in the peritoneal cavity.]
Complications of shunting
Infection: results in meningitis, peritonitis or inflammation
extending along the subcutaneous channel. With a V-A shunt,
bacteraemia may lead to shunt ‘nephritis’. Staphylococcus
epidermidis or aureus are usually involved, with infants at
particular risk. Minimise the risk of infection with prophylactic
antibiotics and in neonates, with antibiotic impregnated shunt
systems. When established, eradication usually requires shunt
removal.
Subdural haematoma: ventricular collapse pulls the cortical
surface from the dura and leaves a subdural CSF collection or
tears bridging veins causing subdural haemorrhage. The risk
may be reduced with a variable pressure or programmable valve.
Shunt obstruction: blockage of the shunt system with choroid
plexus, debris, omentum or blood clot results in intermittent or
persistent recurrence of symptoms. Demonstration of an increase
in ventricular size compared to a previous baseline CT scan
confirms shunt malfunction. Over a third require revision within
1 year and 80% within 10 years.
Low pressure state: following shunting, some patients develop
headache and vomiting on sitting or standing. This low pressure
state usually resolves with a high fluid intake and gradual
149
mobilisation. If not, insertion of an antisyphon device or
conversion to a high pressure valve is required.
Third ventriculostomy: Suitable for patients with tri-ventricular
hydrocephalus e.g. obstructive hydrocephalus caused by
aquaduct stenosis or a pineal or posterior fossa tumour
occluding the posterior end of the 3rd ventricle/aqueduct. By
using a flexible or rigid endoscope introduced through a frontal
burrhole, a fistula is created in the floor of the 3rd ventricle. This
provides an alternative method of treatment, which if successful,
avoids the above problems of shunt insertion. About 2⁄3 of
patients obtain permanent benefit.
150
CHAPTER 18
GUILLIAN BARRE SYNDROME
GUILLAIN BARRÉ SYNDROME (ACUTE INFLAMMATORY
DEMYELINATING POLYNEUROPATHY)
Aetiology/pathology
The condition may follow viral infection, e.g. varicella-zoster,
mumps and cytomegalovirus. It is also associated with
Mycoplasma, Campylobacter, infections, immunisations with
both live and dead vaccines, antitoxins, trauma, surgery and,
rarely, malignant disease and immunodeficiency. Both antibody
and cell-mediated reactions to peripheral nerve myelin are
involved. Some patients produce antibodies to myelin
glycoproteins or gangliosides, others develop a T cell mediated
assault on myelin basic protein. Segmental demyelination results
with secondary axonal damage if the process is severe.
Perivascular infiltration with lymphocytes occurs within
peripheral nerves and nerve roots. Lymphocytes and
macrophages release cytotoxic substances (cytokines) which
damage Schwann cell/myelin. When axon damage and nerve cell
death occur, regeneration cannot take place.
Clinical features
Sensory symptoms predominate at the beginning with
paraesthesia of the feet, then hands. Pain, especially back pain,
is an occasional initial symptom. Weakness next develops this
may be generalised, proximal in distribution or commence
distally and ascend. Tendon reflexes are absent or depressed. In
severe cases, respiratory and bulbar involvement occurs.
Weakness is maximal three weeks after the onset.
Tracheostomy/ventilation is required in 20% of cases. Facial
weakness is present to some extent in 50% of cases.
Papilloedema may occur when CSF protein is markedly elevated
(blocked arachnoid villi?). Autonomic involvement – tachycardia,
fluctuating blood pressure, retention of urine – develops in some
cases. Variants are common (20% of cases).
151
Acute motor axonal neuropathy (AMAN) – often after
campylobacter infection
acute motor, sensory axonal neuropathy (AMSAN)
Investigations
CSF protein is elevated in most patients but often not until the
second or third week of illness. Cells are usually absent but in
20% up to 50 cells/mm3 may be found.
Ancillary investigations
Performed to identify any precipitating infection: e.g. viral and
bacterial studies. Electrolytes are checked for inappropriate
secretion of antidiuretic hormone and immune complex
Diagnosis is based on clinical history supported by CSF and
neurophysiological investigation and exclusion of acute spinal
cord disease, porphyria and myasthenia gravis. Some antibodies
have been identified as being associated with some sub-types
including:
AMAN: anti-GD1a and GM1
Acute sensory neuropathy: anti-GD1b
Treatment
Supportive care in HDU/ITU with prevention of respiratory and
autonomic complications provides the best chance of a
favourable outcome. Signs of impending respiratory failure forced
vital capacity (FVC) below 18 ml/kg, arterial PaCO2 > 6.5 kPa
and PaO2 < 8 kPa on oxygen – indicate elective intubation for
ventilation. When respiratory assistance is likely to exceed 2
weeks, tracheotomy should be performed. Subcutaneous low
molecular weight heparin with support stockings must be given
where the degree of immobility makes thromboembolism a
possible complication. Both plasma exchange (PE) and
intravenous immune globulin (IVIG), 0.4 g/kg daily for 5 days –
are equally effective at speeding recovery and improving outcome.
IVIG is the preferred treatment because of ease of administration
152
but is not without side effects (flu-like symptoms, vasomotor
instability, congestive cardiac failure, thrombotic complications
strokes and myocardial ischaemia, transient renal failure and
anaphylaxis. There is a very small risk of infection, including
theoretically variant CJD) Treatment is generally given to those
who can no longer walk and is deferred in milder cases. Steroids
are not indicated, two trials showing no benefit.
Outcome
Mortality – 2%. Of those progressing to respiratory failure, 20%
are left severely disabled and 10% moderately disabled. In milder
cases the outcome is excellent. Recurrence – 3%. Miller Fisher
variant of Guillain Barré The Miller Fisher syndrome consists of
ophthalmoplegia, areflexia and ataxia without significant limb
weakness. Serum IgG antibodies to a specific ganglioside are
characteristic (anti-GQ1B antibodies). Management is that of
Guillain Barré.
Occupational Therapy Applied in Neurological Conditions.
Course Code: OCT 231: 3 Units Course.
Sensory Modulation
Dr. Ayres (1979, 2005) proposed that the “combination of
facilitatory and inhibitory messages produces modulation, which
153
is the nervous system’s process of self-organization” Sensory
modulation disorder is one type of SI dysfunction characterized
by fluctuating or extremes in the responsiveness to the intensity
of one or more sensations. Difficulties with sensory modulation
are often observed during everyday activities such as grooming,
social events, or transitions. Specific behaviors may include
auditory hypersensitivity or tactile defensiveness, gravitational
insecurity, aversion to movement, or feeling over- whelmed in
high-stimulus environments such as shopping malls (Koomar,
1995; Weisberg, 1984). Sensory modulation disorders are seen in
conjunction with arousal, external regulation, or self-regulation
issues, including colic and poor rhythmic respiration, digestion,
and elimination; arousal and attention deficits; hyperactivity;
anxiety and other signs of emotional instability; and social
problem.
Sensory Discrimination
Sensory discrimination is the interpretation of sensory
information that allows you to know efficiently and accurately
where your body is, where other people are, and details about the
environment from multiple sensory channels. Sensory
discrimination disorders are a result of slow and inaccurate
processing of one or more types of sensory information, under
responsiveness to sensation, inadequate perception formation,
and poor sensory associations. Patterns of dysfunction that have
been identified through factor and cluster analyses indicate that
poor vestibular proprioceptive processing is often associated with
poor anticipatory and reactive postural control and inadequate
bilateral integration and sequencing. Tactile discrimination
deficits are often associated with poor fine motor skills and
praxis. It is as if the child is trying to do precise work with his or
her hands while wearing winter gloves. Visual perceptual deficits
are associated with poor visual construction ability and visual
motor skills. Auditory perceptual deficits often contribute to poor
auditory language skills.
Praxis
Praxis is the ability to conceptualize, plan, and execute skilled
tasks. It underlies engagement in purposeful activity. Any non-
habitual motor task that the child needs to think through
requires praxis. The child who is learning how to dive needs to
154
stand at the water’s edge, get the arms in position over the head,
shift the weight forward, tuck the chin against the chest, and
then maintain this position as he or she falls head first into the
water. All of this requires cognitive effort as well as physical
coordination. Dr. Ayres (1972) hypothesized that sensory
discrimination, particularly of tactile, vestibular, and
proprioceptive sensations along with visual input, was the
foundation for praxis. The resulting sensation from the activity is
processed by the brain, and this further informs individuals
about their body in relation to itself as well as to other people and
objects in the environment (Ayres, 1989, 2004). Through her
factor analyses, she later found a consistent relationship,
particularly between the tactile system and praxis. There are a
variety of different types of praxis dis- orders, including poor
ideation of creative or novel activities (e.g., the child in the
sandbox who shovels sand into the pail and then pours it out
and repeats the process but does not come up with the idea that
she can “make a cake” by filling the pail with sand and then
turning the pail over to form a mound that she can then
decorate); somatodyspraxia, or poor use of the body to motor
plan action sequences; poor use of language for sequencing and
planning; poor ability to modify an action while in motion to
enhance skill and precision; poor visual construction (e.g.,
difficulty replicating a block design); and poor ability to organize
behavior in future time and space. Conceptualizing an action, or
forming the idea about what one wants to do, is a critical aspect
of praxis. This is ideation, and it is a cognitive function (May-
Benson, 2001). Planning how one intends to engage in the task is
also cognitive. A child observing a newly constructed playground
with swings, slides, tree house, poles, and sand will have 101
ideas about what to do and will want to do them all. The actual
execution of the activity is the part of praxis that we can observe,
and this is the part that is assessed to determine a child’s skill
for motor tasks. The child then sequences these ideas into
swinging, sliding, and digging activities and modifies the
sequence or the challenge so that it is more fun and successful.
ASSESSMENT
The process of sensory integration cannot be observed directly,
which is why Dr. Ayres used the term “hidden disabilities” when
referring to SI dysfunction
155
INTERVENTION PLANNING
Occupational therapy using a sensory integrative approach is
guided by the evaluation data. On the basis of the information
from the occupational profile, the therapist con- siders the
identified occupationally related outcomes that are important to
the client, the family, and the reimbursement agency. The
analysis of performance provides detailed information on client
factors, performance skills, and pat- terns that contribute to the
development of client-directed interventions, therapeutic
activities, and environmental modifications. Additionally,
education and consultation are required so that the team
understands the relationship of the child’s sensory integrative
functions to participation in daily life activities. Therapeutic
activities to address the identified deficits are designed with
specific attention to the contribution of the tactile, proprioceptive,
and vestibular sensations to function (Figures 59.5, 59.6, and
59.7).
Special consideration is given to including proprioception in
the form of active movement and heavy work activities.
Proprioception exerts a regulatory influence on other
sensations (Blanche & Schaaf, 2001).
Vestibular activities are especially important so that the
child can develop the capacity to hold the body upright
against gravity while holding still and while moving (Ayres,
1972). Processing vestibular information is important for
the development of the sense of space and navigation
(Berthoz, 2000).
Tactile information is essential for refined interactions with
the external social and physical environment (Ayres, 1972,
2005; Montegue, 1986).
INTERVENTION
Central to intervention using sensory integration principles is a
unique philosophy that reflects Dr. Ayres’ sense of trust,
compassion, and respect for children (Spitzer & Smith Roley,
2001). She proposed that intervention delivered in a playful style
at the child’s level could elicit the child’s “inner drive” to learn
and develop. we facilitate enhanced neuronal growth and
development that lead to increased skill and independence in
156
daily life activities (Ayres, 1972). The therapeutic environment
designed by Dr. Ayres was unique in its ability to safely provide
opportunities for vestibular, proprioceptive, and tactile
sensations and adaptive motor responses. She used simple and
readily available objects such as tires, ropes, wood, hula hoops,
and rocker boards and created unusual obstacle courses and
games with them. She used ceiling beams and devices so that she
could suspend equipment that allowed the child to swing safely
through space. She invented this equipment as the need arose for
individual clients. Although a clinic filled with ceiling hooks,
swings, mats, carpeted barrels, scooter boards, ramps, and
pillows is commonly associated with pediatric occupational
therapy using “classic” SI intervention methods, the environment
alone is not sufficient to define intervention using sensory
integration theory. Dr. Ayres described not only the environment,
but also what might be considered the therapeutic alliance.
Parham and her colleagues (2007) report that intervention using
sensory integration principles should be faithful to the methods
and principles that are considered essential and distinctive to
this particular therapeutic method. The authors examined the
literature and gathered expert opinion to specify the essential
and distinctive sensory integration methods and principles. By
using these principles, a therapist is demonstrating fidelity to the
intervention. Classic SI intervention comprises both structural
processes (e.g., length of the session, therapist’s training,
equipment used) and therapeutic processes (e.g., therapist
creates an environment that invites play and ensures safety,
fosters a therapeutic alliance). Establishing fidelity helps to
clearly delineate the qualities that must be present to define the
intervention as sensory integration, and for the practitioner, it
clearly articulates the specific strategies and process that are
linked to an underlying theoretical base and supporting evidence.
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The use of sensory opportunities that feature varied and
appropriate vestibular, tactile, and proprioceptive
sensations, including thick mats, large overstuffed pillows,
swings, ramps, ladders, ropes, targets, manipulatives, balls,
vibrating toys, various textures such as stretchy soft or
furry fabrics, brushes, props, and materials that are used
during daily routines.
Opportunities to move through space so that the child can
achieve increasingly complex somatomotor adaptive
responses.
The therapist’s scaffolding of success emotionally,
physically, cognitively, and socially.
Facilitating the just-right challenge: The therapist
collaborates with the child on choice and sequence of
activities and equipment and adjusts the intervention
accordingly to ensure success.
Providing environmental affordances (the opportunities that
the environment affords children to do things) that invite
interactions with the environment, including space to move,
jump, and crash and items that stimulate creativity and
engagement.
Assisting in organization of behavior relative to physically
interacting with objects and people in time and space,
including the opportunity to rearrange the environment and
the way in which it is used (Parham et al., 2007).
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Basic Tenets of Sensory Integration
Following are the classic principles of intervention using a
sensory integration approach:
Integrated sensation is “nourishment for the brain”(Ayres,
1979).
Adaptive responses are required to successfully meet
challenges essential for growth and development (Ayres,
1972). The adaptive response is essential to increased
sensory integration.
The inner drive of the human being invites the experience of
life. It is this motivation to enjoy life that Dr. Ayres wished
to engage during intervention.
Active participation promotes organization.
Artful vigilance is essential on the part of the therapist to
facilitate the just-right challenge.
When using sensory integration intervention strategies, the
practitioner will provide a balance of structure and freedom so
that the child has opportunities to problem-solve and make some
of his or her own choices. Some essential characteristics that
differentiate sensory integration methods from other frames of
reference are freedom within the structure of a sensory-rich
environment, the ability to physically move through space and
move objects in space, and the assistance of the practitioner so
that the child can learn to use his or her body in new and novel
ways so that increasingly complex possibilities for physical
engagement emerge. Sensory integration is not sensory
stimulation, due to the imperative of the adaptive response and
modification of the activity based on the child’s reaction
(Anzalone & Murray, 2002). It is not adult planned and designed;
rather, the adult sets up the structure and possibilities in which
the child’s interests and ability to cope with the sensory, motor,
and organization demands dictate the level of challenge and
intensity of the activities. Therefore, the equipment does not stay
in the same place each time, and the therapy does not follow an
orderly and predictable sequence, so each session is somewhat
novel. The intervention is not provided with the expectation of a
subsequent reward. The activities are intended to be fun and
inviting and therefore are rewarding in and of themselves. Play is
one of the most important and powerful parts of the process of
intervention using sensory integration strategies and one that
facilitates the intrinsic reward of this kind of occupational
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engagement (Bundy, 2002). Sensory integrative dysfunction
commonly occurs in children with diagnoses such as autism,
fragile X syndrome, and cerebral palsy. These children will
benefit from a variety of approaches in addition to sensory
integration methods (Mailloux, 2001; Mailloux & Smith Roley,
2004; Schaaf & Smith Roley, 2006; Smith Roley et al., 2001).
During a typical occupational therapy session, sensory
integration methods are often used in conjunction with
complementary methods such as neurodevelopmental treatment
(Blanche, Botticelli, & Hallway, 1995), play- based approaches
(Burke & Mailloux, 1997; Knox & Mailloux, 1997), developmental
and behavioral approaches (Anzalone & Murray, 2002), and
cognitive approaches such as the Alert Program for Self-
Regulation (Williams & Shellenberger, 1996). This is especially
true for children with multiple impairments.
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CHAPTER 19
OCCUPATIONAL THERAPY ROLE WITHIN A MULTI
DISCIPLINARY TEAM
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CHAPTER 20
THE PROCESS OF COGNITIVE REHABILITATION
EVALUATION
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OVERVIEW OF INTERVENTION APPROACHES
Interventions for people with cognitive dysfunction differ in the
areas that are targeted for intervention and in the underlying
assumptions about individuals’ abilities to learn and generalize
information. The characteristics and underlying assumptions of
the different intervention approaches are explored in this section.
Factors that are critical in the selection of these intervention
approaches as well as methods for systematically integrating
them are also discussed. As you read this section, review the
case study on cognition and performance context.
Adaptation
The Cognitive Disability Approach Allen (1985, 1993) designed a
cognitive disability approach that provides guidelines for
matching and adapting the individual’s cognitive level with
activity demands. This approach for evaluation and intervention
was designed initially for people with psychiatric disorders and
chronic cognitive disabilities (e.g., dementia) and later was
expanded for individuals with different central nervous system
problems resulting in cognitive impairments (e.g., stroke). In this
model, function is organized into six ordinal levels of global
function ranging from normal (level 6) to profoundly disabled
(level 1). Modes of performance within each level further qualify
behavior variations and allow for more sensitive measurement of
the person’s capacity. To get an elaborated description of the
Allen Cognitive Level, refer to Allen (1985) and Levy and Burns
2005). Each of Allen’s cognitive levels has three components:
attention, motor control, and verbal performance. The person’s
cognitive progress can be seen as a continuum along two
dimensions, motor and verbal performance, which are linked by
attention. The six cognitive levels are used to describe functional
profiles of capacities and limitations that help to clarify and
direct interventions and care. Allen’s model provides clinicians
with intervention guidelines for different levels of cognitive
function. For example, the cognitive levels identify the required
assistance needed, as well as what the person is still able to do
safely. This information is used to educate and train caregivers
and to make recommendations for assistance and environmental
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adaptations to optimize function and safety. It is also used to
select activities that match the person’s cognitive level and
maximize participation in meaningful occupations (Allen, 1985;
Allen et al., 1992; Levy & Burns, 2005).
Compensation
Compensation teaches the person to bypass or minimize the
effects of the impairment by modifying the method that is used to
perform an activity. The client is expected to initiate and
implement the use of an external aid or strategy to enhance
occupational performance in a variety of different situations
(AOTA, 1999). The complexity of the task and the compensatory
strategy may require awareness and acceptance to be generalized
and independently applied to a variety of situations (Birnboim &
Miller, 2004; Toglia, 1993a). For example, a memory notebook
could be used to compensate for memory loss. Independent use
requires that the person recognize that he or she is having
difficulty with memory and perceive the need to write things
down to aid in remembering. It also requires initiation of use of
the book in multiple situations. Clients with more severe
cognitive impairments can be trained, through rote repetition and
errorless learning, to use simplistic compensatory strategies and
modifications with minimal awareness of why the strategy is
needed (Giles, 2006; Hallgren & Kottorp, 2005; Kottorp et al.,
2003). In these clients, the compensatory strategy becomes
automatic and integrated into the person’s habits or functional
routines.
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CHAPTER 21
DISEASE OF THE SPINAL CORD
MULTIPLE SCLEROSIS
PREVALENCE/INCIDENCE
Approximately 400,000 Americans have MS, and every week,
about 200 people are diagnosed. Worldwide, MS may affect 2.5
million individuals. Most people with MS are diagnosed between
the ages of 20 and 50 years. It affects twice as many women as
men.
COURSE
People with MS can expect one of four clinical courses of disease,
each of which might be mild, moderate, or severe (National
Multiple Sclerosis Society, 2004).
Relapsing-Remitting
In the relapsing-remitting course, the person experiences clearly
defined exacerbations (relapses) and episodes of acute worsening
of neurological function that are followed by partial or complete
recovery periods (remissions) free of disease progression. This is
the most common form of MS at time of initial diagnosis (∼85%).
Primary Progressive
In the primary-progressive course, the person experiences a slow
but nearly continuous worsening of the disease from the onset,
with no distinct relapses or remissions. However, there are
variations in rates of progression over time, occasional plateaus,
and temporary minor improvements. This course is relatively rare
(∼10%).
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Secondary-Progressive
In the secondary-progressive course, the person experiences an
initial period of relapsing-remitting disease followed by a steadily
worsening, unpredictable disease course. About 50% of people
with relapsing-remitting MS developed this form of the disease
within 10 years of their initial diagnosis. However, there is
preliminary research to suggest that disease-modifying
treatments may significantly delay this progression.
Progressive-Relapsing
In the progressive-relapsing course, the person experiences a
steadily worsening disease from the onset but also has clear
acute relapses, with or without recovery. In contrast to relapsing-
remitting MS, the periods between relapses are characterized by
continuing disease progression. This course is relatively rare
(∼5%).
PROGNOSIS
According to Dirette (2007), approximately 60% of individuals
with MS can continue to be fully functional for up to 10 years
following their first exacerbation. In addition to this,
approximately 30% of individuals with MS continue to be able to
function 30 years after their first exacerbation. The fact that an
individual is diagnosed with MS does not mean that his or her
life expectancy is significantly decreased. There are some
individuals, however, who do become quite disabled secondary to
MS, and some even die prematurely from infections or
complications (Dirette, 2007).
SYMPTOMS
Primary Signs as a Result of Demyelination of the CNS
Muscle stiffness (spasticity) or weakness
Tremulousness in extremities
Paresthesias, numbness, and blunting of sensation (e.g.,
reduced pain or temperature sense, disturbances of
vibratory or position sense)
Partial or complete paralysis of extremities
Visual deficits (vision loss, blurred or double vision)
Extended periods of fatigue
Interruptions in sexual functioning
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Problems with memory, concentration, information
processing, or rapid problem solving skills
Visual-spatial deficits
Difficulty articulating speech
Fluctuation in mood/depression
Emotional lability
Secondary Signs
Pain
Staggering gait and/or loss of balance
Poor coordination
Loss of bladder or bowel control; frequent urinary tract
infections
Anxiety and sleep disturbances
Contractures and pressure sores
Decreased ADL skills
Tertiary Signs
Shift in roles, changing responsibilities
Social isolation
Divorce
Loss of independence
Stigma of disability (Shapiro, 2003)
INTERDISCIPLINARY TREATMENT
Medications
Corticosteroids are used to shorten acute attacks, reduce
inflammation, and ease symptoms.
Disease-modifying treatment with injections of the protein
interferon-beta reduces the frequency of re- lapses in MS
and might help to delay eventual disability. These
medications have shown effectiveness in modifying the
natural course of relapsing MS by altering the rate and/or
extent of disease progression. Medication management with
these “disease modifiers” is recommended as early as
possible for individuals with a relapsing course.
More potent medications are being developed that are
effective in slowing down MS that is rapidly worsening or
becoming progressive (Beers & Berkow, 1999).
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Rehabilitation
Physical therapy: Physical therapy focuses primarily on
mobility and the use of mobility aids, spasticity of the
muscles, and physical fitness. Personalized exercise
programs may help people recover muscle control and
strength after an exacerbation. There is significant evi-
dence associating aerobic exercise with improved quality of
life, mobility, endurance, and reduction in fatigue (Mostert
& Kesselring, 2002). Hydrotherapy is a popular form of
aerobic exercise that addresses the fatigue and weakness
experienced by individuals with MS.
Occupational therapy: Occupational therapy focuses on
strength, coordination, and fine motor control of the upper
extremities. Fatigue and pain management tech- niques
have been well supported as a means to improve
occupational performance and satisfaction. Psycho- social
intervention is embedded in the treatment plan to address
cognitive, emotional, and self-concept issues.
Speech therapy: In progressive forms of MS, problems with
speech or swallowing due to muscle weakness or a lack of
coordination may need to be addressed.
Psychosocial support/counseling: Individual or
grouptherapy can help individuals with MS and their
families to deal with depression, anxiety, and the
unpredictability of the disease process. Evidence suggests
that a personalized psychosocial rehabilitation program
encourages active participation, increased autonomy, and
improved quality of life (Ferriani et al., 2002).
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OT AND THE EVIDENCE
Meta-analysis suggests that occupational therapy–related
treatments are effective in treating the deficits associated with
MS, particularly for outcomes in the capacity and ability (e.g.,
muscle strength, ROM, mood) and task and activity (e.g.,
dressing, bathing, ambulation) levels of performance. The effect
sizes for the capacities and abilities outcomes were generally
large for studies that examined a specific intervention method,
such as exercise, fatigue management, cooling, and
transcutaneous electrical nerve stimulator application for pain. A
smaller effect size has been shown for emotional and cognitive
outcomes, such as stress management, skills training in
socialization, and attention training (Baker & Tickle-Degnen,
2001). Alternative therapies including acupuncture, massage,
yoga, meditation, and dietary modifications have shown
encouraging results as part of the treatment program for
individuals with MS (MS Australia, 2001). Fatigue is one of the
most common targets of OT intervention. It impedes an affected
person’s ability to fully engage in desired occupational
performance and roles. There is significant evidence to support
the use of energy conservation education within both community
based and inpatient rehabilitation settings to reduce the impact
of fatigue among people with MS and possible positive change
associated with peer support (Vanage, Gilbertson, & Mathiowetz,
2003). Energy conservation courses, including education about
rest and delegation of tasks to others, have been found to be
effective intervenetions (Holberg & Finlayson, 2007; Matuska,
Mathiowetz, & Finlayson, 2007; Vanage et al., 2006). Coping
involves cognitive and behavioral efforts to master, reduce or
tolerate an external or internal demand created by a stressful
situation. Certain types of coping strategies have been shown to
be associated with better or worse adjustment in MS. Many
studies in the chronic illness literature have demonstrated that
high levels of increasing feelings of self-efficacy, improving quality
of life, and lower levels of depression are associated with
emotion-focused coping. In contrast, problem-focused coping has
been shown to be associated with lower levels of depression and
higher levels of adjustment. OT intervention should involve
teaching successful coping and integrating it into daily life
(Artnett, Higginson, Voss, Randolph, & Grandey, 2002).
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CAREGIVER CONCERNS
MS affects people in their most productive years: young adults
readying themselves to leave home in pursuit of academic,
vocational, or social goals; men and women starting their careers
and families of their own; and those in middle age who are
enjoying their productive years and planning for retirement. In
each of these age groups, the diagnosis of MS has a significant
impact on the individual and the family members and/or loved
ones in his or her life. Challenges to family coping include the
following:
Individual needs and coping styles
Disruption in family rhythm
Disruption in family communication
Uncertainty and anxiety
Adaptation and adjustment
Impact of cognitive changes
Approximately 100,000 people who have MS require help with
daily activities or personal care. They receive most of their help
from spouses, who typically have major additional
responsibilities, including employment and child care. Caregiver
burnout has many different causes, including physical strain and
emotional stress. Some ways to address these issues include the
following:
Effective communication
Relieving pressures of caregiving
Planning and decision making for the future (Kalb.
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