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CEREBRAL PALSY ● Untreated jaundice

- Group of permanent disorders of the development of ● Severe neonatal infection


movement & posture
- Causes activity limitations that are attributed to POSTNEONATALLY Acquired CP
nonprogressive disturbances that occurred in the Metabolic Encephalopathy
developing fetal or infant brain ● Storage disorders
● Intermedullary metabolism disorders
Motor Disorders ● Metabolic disorders
- Often accompanied by disturbances of sensation, ● Misc. disorders
perception, cognition, communication and behavior as ● Toxicity (alcohol)
well as seizures and secondary musculoskeletal Infections
problems ● Meningitis
● Septicemia
Comorbidities in ages 5-8 y/o: ● Malaria (developing countries)
40% ➜ mental retardation Injuries
75% ➜ slow learners, persons w/ learning disabilities ● Cerebrovascular accident
35% ➜ epilepsy ● Following surgery for congenital malformations
20% ➜ visual impairment ● Near-drowning
9% ➜ hydrocephalus ● Trauma
13% ➜ combo of 2 impairments ● Motor vehicle accident
15% ➜ combo of 3 impairments ● Child abuse such as shaken baby syndrome
25% ➜ difficulty with speech
25% ➜ hearing impairments
40-50% ➜ increased frequency of visual impairments

Diagnosed with CP if insult occurred:


a. Prenatally (75%)
- Majority of CP in developing countries
- Not associated w/ significant neonatal
encephalopathy
b. Perinatally (6-8% ➜ perinatal asphyxia)
c. Postnatally (10-18%)

*Diagnosed w/ CP throughout infancy and early childhood

ETIOLOGY
- No single specific cause
- Potential causes:
● Occur in prenatal stage of development
● Grouped with congenital problems in the perinatal /
neonatal, and in postnatal / postneonatal time period

PRENATAL Causes of CP
● Vascular events such as a middle cerebral artery
infarct
● Maternal infections during the first and second
trimesters such as rubella, cytomegalovirus,
toxoplasmosis
● Less common: metabolic disorders, maternal
ingestion of toxins, and rare genetic syndromes

PERINATAL Causes of CP
Problems During Labor and Delivery
● Obstructed labor
● Antepartum hemorrhage
● Cord prolapse
Other neonatal causes
● Hypoxic-ischemic encephalopathy
● Neonatal stroke (usually middle of cerebral artery)
● Severe hypoglycemia
Congenital Problems that result in CP (Infant & child)
1. Schizencephaly
- Segmental defect that causes cleft in brain
2. Lissencephaly
- Defect in neuronal migration that normally
goes toward the periphery of the brain
- Results in smooth brain
- Aka decreased cerebral gyri
3. Microcephaly & megalocephaly
4. Cortical dysgenesis
- Disorder of brain cortex formation
- Defects in the normal formation and
remodeling of synapses

*Approx. Half of created neurons die off (apoptosis) during


MIDGESTATION
*axons and synapses are also eliminated during first 10 YRS
or more of normal development
*neural elements that best persist = fit the environment
*changes in formation of developing NS = infant with CP

➜ Plasticity / Equipotentiality
- Greater ability of uninjured part to assume the
function of injured part of brain
- Immature brain has more of this
- Response to injury is different & makes diagnosis &
prognosis difficult

DIAGNOSIS & PROGNOSIS


● Signs & symptoms apparent in EARLY INFANCY
● Infants may be diagnosed earlier than 2 y/o when:
○ Abnormal muscle tone
○ Atypical posture
○ Movement w/ persistence of primitive
reflexes
● Milder cases of CP = diagnosed 4-5 y/o
● Evaluation
○ Motor skills
○ Neuroimaging (cranial US, CT, MRI) - show
loc & type of brain damage
○ Evidence that symptoms are not progressing
● Clinical findings + neuroanatomy

➜ Neuroimaging
1. Cranial ultrasound
- High-risk preterm infant
- Less invasive
2. Computed tomography
3. Magnetic resonance imaging
- Preferred
- Provides greater detail of brain tissue &
structure
- 70-90% with CP have abnormalities here

➜ Neuroimaging is unremarkable:
● Mitochondrial & metabolic disorders
● Transient dystonia
➜ Cerebral hemorrhages ◊ indicated that intellectual disability was the single strongest
- Assoc. w/ CP predictor of survival of the child with CP (profoundly mentally
● Intraventricular hemorrhage (IVH): Bleeding into retarded
tissue around ventricles children with CP do not live into adulthood) and that the
● Germinal matrix hemorrhage (GMH): bleeding into second most important factor impacting life expectancy was
tissue around ventricles the severity of the physical impairments.
● Periventricular intraventricular hemorrhage (PIVH): ◊ the life expectancy of children with CP and found, from
bleeding into both areas multiple sources, that the causes of mortality related most
● Periventricular cyst (PVC): may form in same areas commonly to the respiratory and circulatory systems, certain
as acute hemorrhage resolves cancers, and neurologic complications.
- Known risk factors: mechanical ventilation & injury
during critical periods of brain dev’t
CLASSIFICATION OF CP
◊ There are known risk factors for hemorrhages including Type of Movement Disorder
mechanical ventilation and injury during critical periods of brain 1. Spastic
development. 2. Hypotonic
3. Dyskinetic
◊ Notably, periventricular white matter is most sensitive to insult 4. Ataxic
and injury between 24 and 34 weeks of gestation.
◊ Hemorrhages are graded in increasing severity from I Anatomical Location of Limbs Affected
through IV. 1. Hemiplegia (one arm & leg ipsilaterally)
◊ The grade of bleed alone cannot predict the development or 2. Diplegia (both LEs)
severity of CP. 3. Quadriplegia (all four limbs + back, neck)
◊ Palmer indicated that cranial ultrasound should be used for
low-birth-weight infants to detect grades III and IV Scope of Motor Dysfunction
hemorrhages, IVH, cystic periventricular leukomalacia (PVL), ● Gross Motor Function Classification System (GMFCS)
and ventricular enlargement - b/w ages 6 & 12
◊ After term age, cranial ultrasound and MRI are used to
identify cystic PVL and ventriculomegaly, which are associated
Level I Walks w/o restrictions
with subsequent development of CP. Limitation in more advanced gross motor skills
◊ PVL is the major cause of CP in infants born preterm.
◊ The extent and location of white matter damage can lead to Level II Walks w/o assistive devices
different subtypes of CP. Limitations in walking outdoors & in community
◊ Localized damage to the cortical spinal tracts often results in
spastic diplegia, and when the lesions extend laterally, Level III Walks w/ assistive mobility devices
quadriplegia is often the result. Limitations in walking outdoors & in community
◊ It is understood that a neurologic examination alone is
Level IV Self-mobility w/ limitations
insufficiently
Children are transported or use power mobility
sensitive enough or specific enough for early detection of CP. outdoors & in community

◊ The quality of an infant’s “general movements” (GMs) have Level V Self-mobility is severely limited even w/ use of
been used by doctors and researchers to evaluate brain assistive technology
function.
◊ A predominance of cramped synchronous GMs and absence - developed to fill the need to have a standardized
of normal fidgety movements of limbs, neck, and trunk were system to measure the “severity of movement
predictive of CP at 2 to 3 years of age with a sensitivity of disability” in children with CP
100% and a specificity of 92.5% to 100%. ● Level I describes the child with the most independent
◊ Clearly, abnormal GMs at the fidgety age (2 to 4 months function, where he or she can perform all the activities
postterm) of his or her age-matched peers, albeit with some
implies a total absence of the elegant, dancing complexity of difficulty with speed, coordination, and balance.
fidgety movements and predicts CP with an accuracy of 85% ● Level V describes the child who has difficulty
to 98%. controlling his or her head and trunk posture in most
◊ GMs can be predictive of later CP and are the best positions and in achieving any voluntary control of
expression of functional motor development. GMs are movement
analogous to later functional motor milestones and may also
predict severity, as the earlier the GMs are recognized, the
more severe the later limitations in motor function.
◊ Others have found cognitive abilities to be linked with the
severity of CP and to be predictive of many outcomes.
✦ Spastic CP - a discrepancy between upper extremity (UE) and LE
- Approx. 75% of children with CP function in children with this form of CP, with the LEs
- Most common neurologic abnormality being more affected than the UEs and trunk
● 40% or 44 ➜ quadriplegia - Gait deficits such as equinus and crouched gait
● 17% or 33 ➜ diplegia posture tend to be the area of greatest concern for
● 21% or 23 ➜ hemiplegia these children
- Hypertonia in which resistance to passive movement - Owing to bilateral LE spasticity and weakness, energy
increases with increasing velocity of movement expenditure is much greater during ambulation,
resulting in poor endurance and decreased functional
MODIFIED ASHWORTH SCALE mobility at home and within the community.
- Children with diplegia generally have normal cognition
Score Description of Muscle Tone
but may have some social and emotional difficulties
00 Hypotonia - Children with diplegia often require assistive devices
such as a posterior walker, or lofstrand crutches
0 Normal tone, no increase in tone - A scooter or wheelchair may be necessary for
long-distance mobility
1 Slight increase in tone manifested by a slight catch
and release or minimal increased resistance to joint ✦ Hemiplegia
range of motion - subtype of spastic CP in which the child’s upper and
lower extremity on the same side of the body are
1+ Slight increase in tone manifested by a slight catch
& minimal increased resistance to joint ROM for affected
more than half the joint range
◊ Four main types of brain lesions result in hemiplegic CP
2 More marked increase of tone through most of the 1. Periventricular white matter abnormalities have been
whole joint range, but the affected joint is easily reported as the most common diagnostic finding in
moved children with hemiplegic CP
2. Cervical–subcortical lesions, brain malformations, and
3 Considerable increase in muscle tone; passive
nonprogressive postnatal injuries have also been
movement difficult but possible
identified as the main causes of hemiplegia
4 Affected joint is stiff & cannot be moved
- UE is typically more affected than the LE, and both
tend to have more distal involvement than proximal
Spasticity involvement.
- Hyperactive stretch reflex - Muscle spasticity on the affected side decreases
- Responsive to variety of treatments: botulinum toxin, muscle and bone growth, resulting in decreased
baclofen, selective dorsal rhizotomy, orthopedic range of motion (ROM)
surgery - children with hemiplegia often present with
- Causes histologic changes: decreased longitudinal contractures and limb-length discrepancies on the
growth of muscle fibers, decreased volume of muscle, involved side
change in muscle unit size, change in muscle fiber - affected side of the child with hemiplegia often
type presents with shoulder protraction, elbow flexion, wrist
- Secondary disorders: hip dislocation, scoliosis, knee flexion and ulnar deviation, pelvic retraction, hip
contracture, torsional malalignments of femur & tibia internal rotation and flexion, knee flexion, and forefoot
- Effects on function: effortful gait patterns, difficulty contact only due to plantarflexed foot
assuming & sustaining seated positioning, difficulty - Children with hemiplegia tend to achieve all gross and
performing self-care activities (toileting, bathing, fine motor milestones but not within the typical time
dressing, self-feeding) frame
- For example, children with hemiplegia tend to walk
✦ Diplegia between 18 and 24 months but present with gait
- Most common form of spastic CP deficits.
- white matter infarct in the periventricular areas - Additionally, acquisition of bimanual hand skills is
caused by hypoxia can lead to spastic diplegic CP delayed because of the neurologic impairment of the
- primarily affects bilateral LEs, resulting in issues with affected side
gait, balance, and coordination - For example, children with hemiplegia are able to cut
- In standing, children with diplegia often present with food using a fork and knife, but only after hours of
an increased lumbar spine lordosis, anterior pelvic tilt, extensive guided practice during occupational therapy
bilateral hip internal rotation, bilateral knee flexion, and at home
intoeing, and equinovalgus foot position - Two widely known standardized assessments are
used to evaluate the quality of UE function in children
with hemiplegia: the SHUEE and the Assisting Hand spatial characteristics; and are usually large motions
Assessment (AHA) of the more proximal joints.
- Cognitive function is typically normal in these children, - Athetosis is rare as a primary movement disorder and
and as adults they are able to work and participate in is most often found in combination with chorea.
a variety of professional settings with spastic - Athetosis is most commonly a secondary movement
hemiplegia have been found to have social and disorder in conjunction with spasticity
emotional deficits. - The cortical–basal ganglia–thalamic loop is a sensory
- These include emotional disorders in 25%, conduct and motor feed-forward and feedback circuit and
disorders in 24%, pervasive hyperactivity in 10%, and when impaired results in athetosis.
situational hyperactivity in 13% - Older individuals with athetoid CP are at risk for
- Overall, children with hemiplegia require minimal acquiring devastating neurologic deficits owing to
equipment or self-care/school accommodations. intervertebral disc degeneration and instability in their
- They may benefit from orthotics, assistive devices cervical spines
such as a cane, adaptive self-care equipment, or - Individuals with athetosis typically initiate and attempt
accommodations due to visual impairments. control of movement with the jaw and head
- This eventually causes musculoskeletal changes such
✦ Quadriplegia as cervical instability, potential high spinal cord
- volitional muscle control of all four extremities is injury, temporomandibular joint dysfunction, and
severely impaired or spinal stenosis
- often accompanied by neck and trunk involvement - when athetosis is the primary movement disorder, the
- Like diplegic CP, periventricular white matter lesions cognitive ability of these children tends to be
are the most frequently observed neuroimaging underestimated owing to associated dysarthria
finding in children with quadriplegic CP - tend to have normal to above-normal intelligence
- Extensive lesions affecting the basal ganglia or - Rigidity is much less common and is felt as
occipital area often lead to visual impairments and resistance to both active and passive movement and
seizures, both commonly seen in children with this is not velocity dependent.
subtype of CP - Tremor, a rhythmic movement of small magnitude,
- Cognition can vary from normal to severely impaired usually of the smaller joints, rarely occurs as an
and is unique to each child with quadriplegia isolated disorder in CP but rather in combination with
- It is important to note that children with quadriplegia athetosis or ataxia
who are unable to speak are often regarded as being - Dystonia is a slow motion with a torsional element
cognitively impaired that may involve one limb or the entire body and in
- once provided a means of effective communication, which the pattern itself may change over time
some are able to express their level of understanding - Ballismus is the most rare movement disorder and
and critical thinking involves random motion in large, fast patterns usually
- Gross and fine motor abilities vary widely for children of a single limb
with quadriplegia, from being ambulatory for - Choreoathetosis involves jerky movement,
household distances with an assistive device to being commonly of the digits and varying in the ROM.
dependent for all care.
- The equipment needs for these children are ✦ Ataxic
considerable through the life span ◊ Ataxic CP is primarily a disorder of balance and control in
- Common equipment recommendations include the timing of coordinated movements along with
mechanical lift systems, wheelchairs, standers, gait weakness, incoordination, a wide-based gait, and a noted
trainers/walkers, feeding systems, bath systems, and tremor.
toileting systems. ◊ This type of CP results from deficits in the cerebellum and
- Home modifications should be considered for children often occurs in combination with spasticity and athetosis.
with severe disabilities to maximize the child’s ◊ The cerebellum is a major sensory processing center, and
independence with transfers and mobility, to ease when impaired, ataxia will result.
caregiver strain, and improve safety for the child and ◊ Children with ataxia have difficulty with transference of skills,
caregiver. and may benefit from a specific task-oriented approach to
treatment.
✦ Dyskinetic ◊ For example, to master stepping onto and off the school bus,
- result in generally uncontrolled and involuntary it is most effective to practice the skill using bus steps.
movement that includes athetosis, rigidity, tremor,
dystonia, ballismus, and choreoathetosis 7. Hypotonic
- Common abnormalities found in imaging include deep ◊ Hypotonia in a child with CP can be permanent but is more
gray matter lesions and, to a lesser extent, often transient in the evolution of athetosis or spasticity and
periventricular white matter lesions might not represent a specific type of CP.
- Athetosis always has involuntary movements that are
slow and writhing; abnormal in timing, direction, and
◊ For example, an infant who presents with generalized
hypotonia through the trunk and extremities will often develop
spasticity beginning distally and progressing proximally.
◊ Hypotonia is typically correlated with congenital
abnormality, such as lissencephaly.
◊ A common mixed tone pattern is seen in some children with
quadriplegia, with spasticity evident in the Les and severe
hypotonia in the trunk and neck.

Assessment of the infant and child with CP


◊ believe that a diagnosis of very mild CP should be possible at
8 months of age.
◊ Identification depends on a combination of suspicious and
abnormal signs revealed during comprehensive assessment
of attainment of motor achievements, neurologic signs,
primitive reflexes,
and postural reactions.
◊ Infants and children with persistent subtle or mild signs
should be monitored closely until the possible outcome is clear.

◊using the Movement Assessment in Infants (MAI), found


certain items that can help distinguish the infant with CP from
the uninvolved infant at 4 months of age.
◊ Items of diagnostic value include neck hyperextension
and shoulder retraction, ability to bear weight on the forearms
while prone, ability to maintain a stable head position
in supported or independent sitting, and the infant’s ability
to flex the hips actively against gravity.

◊ Seven of the 17 MAI items that Harris found highly significant


predictors for CP are observational items.
◊ Both Harris and Milani- Comparetti found that watching the
infant move against gravity is of greater diagnostic value than
intrusive handling or attempts to stimulate a response.
◊ Rose-Jacobs et al.33 evaluated whether the MAI predicted
2-year cognitive and motor development status measured by
the Mental and
Psychomotor Scales of the Bayley Scales of Infant
Development.
◊ They found that the MAI appears to be valid for use with
infants born at term who are at risk of developmental delay.
◊ This test may be a useful tool to help clinicians make
decisions about the provision of intervention services.
◊ In order to understand the atypical movement and motor
control that occurs in children with CP, the therapist must
understand the acquisition of motor control against
gravity, the development of postural control, and the
musculoskeletal development in typically developing
children.
◊ The purpose of the assessment is to discover the functional
abilities and strengths of the child, determine the primary and
secondary impairments (compensations used because of the
primary impairments), and discover the desired functional and
participation outcomes of the child and family.
◊ The therapist must use an organized approach to the
observation of, interaction with, and handling of the child in
order to get an accurate baseline of the child’s functional
abilities.

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