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of Child Neurology

Dystonia in Childhood: Clinical and Objective Measures and Functional Implications

Larissa Pavone, Justin Burton and Deborah Gaebler-Spira
J Child Neurol 2013 28: 340 originally published online 29 June 2012
DOI: 10.1177/0883073812444312
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Topical Review Article

Journal of Child Neurology
28(3) 340-350
The Author(s) 2012
Reprints and permission:
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DOI: 10.1177/0883073812444312
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Dystonia in Childhood: Clinical

and Objective Measures and
Functional Implications

Larissa Pavone, MD1,2, Justin Burton, MD1,2, and Deborah Gaebler-Spira, MD1,2

Abstract
Dystonia is a complex movement disorder that is challenging to identify and quantify. The aim of this article is to review the clinical
scales, kinematic measures, and functional implications of dystonia. Clinical measures include the Barry-Albright Dystonia Scale,
the Burke-Fahn-Marsden Movement Scale, the Unified Dystonia Rating Scale, the Global Dystonia Rating Scale, and the Movement
Disorder-Childhood Rating Scale. The evidence, reliability, and validity of each scale will be outlined. The Hypertonia Assessment
Tool will be discussed emphasizing the importance of discriminating hypertonia. The role of kinematic measures in analyzing
dystonia will be explored, as well as the potential for its future clinical applications.
Keywords
dystonia, hypertonia, kinematics, rating scales
Received January 20, 2012. Accepted for publication March 2, 2012.

Discriminating dystonia in children is complex and requires

careful observation and knowledge of the syndromes commonly associated with hypertonia. Unlike adults, children frequently have a secondary dystonia that is confounded by
accompanying spasticity. This review will highlight the differences in spasticity and dystonia.
Interest in dystonia has increased with the recognition that
many children with cerebral palsy have residual hypertonia
attributable to dystonia, which continues to impact development and function following anti-spasticity procedures. In
addition, response to treatment varies depending on the hypertonia. Accurate differentiation of hypertonia allows specific
treatments targeting dystonia and importantly maximizes outcomes for the child. A clinical tool, the Hypertonia Assessment
Tool (HAT) has been developed to meet the needs of a clinician
in this difficult task of discriminating between spasticity and
dystonia.1
Once the determination of the primary tone abnormality is
established, the task of rating severity is important. The rating
scales that have been utilized in adults and now in children will
be reviewed. Because dystonia represents a hypertonic movement disorder, kinematic measures are emerging as useful
tools. Kinematics allows analysis of fine detail that escapes the
human eye. There are recent protocols developed with upper
extremity kinematics, which will be highlighted.
Importantly, dystonia affects function. Dystonia can be
action-induced or posture-induced; basic functional measures
and developmental assessments provide additional input to
determine the effectiveness of treatments. Dystonia that is a

component of chronic conditions of the nervous system has

implications in all outcome areas of the World Health Organization International Classification of Function (WHO-ICF)
model (Figure 1).2 By combining the tools discussed in this
review, clinicians will best identify impairments of abnormal
tone, severity of the condition, and the abilities of the child.

Definitions/Etiology
Dystonia is defined as a movement disorder in which involuntary sustained or intermittent muscle contractions cause twisting and repetitive movements, abnormal postures, or both.3
Primary dystonias are those conditions in which dystonia is the
dominant manifestation; otherwise, the dystonia is seen as
secondary.4 Dystonic movements are characterized by the
co-contraction of antagonist muscles and overflow of activity
into extraneous muscles resulting in an abnormal pattern of
muscle activation during voluntary movement or the maintenance of posture.5,6
1

Northwestern University, Feinberg School of Medicine/Rehabilitation

Institute of Chicago, Chicago, IL, USA
2
Rehabilitation Institute of Chicago/Northwestern University, Feinberg School
of Medicine, Chicago, IL, USA
Corresponding Author:
Deborah Gaebler-Spira, MD, Rehabilitation Institute of Chicago,
Northwestern University, Feinberg School of Medicine, 345 East Superior St,
Chicago, IL 60611.
Email: dgaebler@ric.org

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Pavone et al

341

Figure 1. WHO-ICF Model, 2002.

Dystonia may be associated with hypertonia, or with

hyperkinetic movements that are excess or unwanted movements.7 It is often difficult to discriminate dystonia. Hypertonia
is defined as abnormally increased resistance to externally
imposed movement about a joint and may be caused by dystonia, spasticity, or rigidity.3 Spasticity is a velocity-dependent
resistance to muscle stretch.3 Once one type of movement or
tone abnormality is treated, another underlying movement may
become more apparent. This frequently occurs with secondary
dystonias such as those seen with cerebral palsy. The Taskforce
on Childhood Movement Disorders has stressed the importance
of consensus definitions for childhood motor impairments.3,7,8
Consistent terminology is important for identification, treatment,
and research of movement disorders.6
The evidence suggests that dystonia may originate in the
basal ganglia and that this may be combined with deficient
inhibition in the motor cortex, brainstem, and spinal cord.5,6
Therefore, dystonia could possibly represent abnormally excessive motor cortex outflow with involuntary activation of muscles
not normally involved in the desired movement or posture.4 An
abnormal cortical sensorimotor integration mechanism was
shown using an electrophysiological approach9-11 and also using
a biokinetic approach.11,12 The thought is that the abnormal
motor cortex outflow would translate into an abnormal pattern
of muscle activation peripherally.

Clinical Measures
Clinical measures are essential for optimal care of the patient
with dystonia. A thorough neurologic examination as well as
a valid and reliable scale assists the clinician in objectively
quantifying dystonia. It is important to choose a scale that
allows the clinician to be consistent and is practical to apply
in a clinical setting. Identification and quantification of the
severity of dystonia is necessary to determine the most valuable
treatment regimen and to monitor the response to treatment.
The activity and function of the child with dystonia should also
be considered.
The Hypertonia Assessment Tool for children was developed to differentiate the subtypes of hypertonia: dystonia,

spasticity, and rigidity.1 Prior to the development of this assessment tool, identifying movement disorders and differentiating
between them was limited to the neurologic examination. The
Hypertonia Assessment Tool is a 7-item clinical assessment
tool designed for children aged 4 to 19 years. An evaluation
using the Hypertonia Assessment Tool should take 5 minutes
or less for each limb and be applied to each limb separately.13
The Hypertonia Assessment Tool consists of 2 spasticity items,
2 rigidity items, and 3 dystonia items (Appendix A). Each of
the 7 items is scored as either present or absent. If 1 subitem
is scored as present, then some form of hypertonia is present.
If 2 or more subitems from different categories are scored as
present then the child has mixed hypertonia. The reliability and
validity of each item in 34 children was examined while developing the Hypertonia Assessment Tool.1 The Hypertonia
Assessment Tool was found to be valid and have substantial
interrater reliability and excellent test-retest reliability for
identifying spasticity (Table 1). For dystonia, interrater reliability was found to be fair and test-retest reliability was moderate. Validity for dystonia ranged from fair to substantial.
Overall, the Hypertonia Assessment Tool was found to be
stronger in identifying the presence of spasticity and dystonia
rather than the absence. For rigidity, the Hypertonia Assessment Tool was better at identifying when rigidity was absent
rather than present. The test-retest, interrater reliability, and
validity were all excellent for identifying the absence of rigidity.1 The Hypertonia Assessment Tool is useful both clinically
and for research purposes to assist in identifying the presence
or absence of dystonia, spasticity, and rigidity.
The Burke-Fahn-Marsden Movement Scale (BFM) was
developed in 1985 to assess primary dystonia.14 This scale was
designed with 2 components: a movement scale based on
examination of the patient and a disability scale (Appendix B).
The movement scale evaluates 9 body regions: eyes, mouth,
speech and swallowing, neck, trunk, right arm and leg, left arm
and leg. Two factors are examined in each region, a provoking
factor and a severity factor. The provoking factor evaluates the
circumstance in which the dystonia appears: 0 equals no dystonia at rest or with action, 2 equals dystonia on particular action,
3 equals dystonia on action of distant part of body or intermittently at rest, and 4 equals dystonia at rest. The severity factor
is rated 0 (no dystonia) to 4 (severe dystonia). The score for
each region is the product of the provoking factor, severity factor, and a weighting factor. The mouth and neck regions are
weighted by 0.5 and the remainder of the regions are weighted
by 1. The neck and mouth regions were designed to carry
decreased weight in the score because they were believed to
contribute less to overall disability.14 Once the product of each
region is obtained, the score is added for a maximal score of
120; the higher the score, the more severe the dystonia. The disability scale is based on the individuals assessment of how the
dystonia affects his or her activities of daily living. The individual rates how his or her speech, handwriting, feeding, eating/
swallowing, hygiene, dressing, and walking are affected by the
dystonia. All of the areas assessed are rated 0 to 4 except for
walking, which is rated 0 to 6. The maximum score on the

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342

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Internal Consistency

PABAK 1.0
PABAK .43
PABAK 1.0

Test-Retest

Abbreviations: ICC, intraclass correlation; IRC, interrater correlation; PABAK, prevalence- and bias-adjusted kappa.

Yes

Yes

Part 1
ICC .98-1.0
IRC .95-1.0
Part 2
ICC .99-1.0
IRC .98-1.0
Part 1
ICC .97-1.0
IRC .95-1.0
Part 2
ICC .99-1.0
IRC .98-1.0

Part 1 Cronbach
a .96 Part 2
Cronbach a .81

Battini et al 2008

Part 1 Cronbach
a .94
Part 2 Cronbach
a .81

Yes

ICC .72

Cronbach a .91

Yes

Yes

Yes

Yes

Clinically
Applicable

Comella et al 2003

Spearman correlation Correlation with Spearman r for

at 2 times .98, .99
ordinal data between
Movement Scale score and 2
alternate ways to assess
dystonia (Global Severity Rank
and Disability Scale score); all
were significant
ICC .967, .978
ICC .978

PABAK .57, .74

PABAK .30, .65
PABAK .91, 1.0

Validity

ICC .71

ICC .866

ICC .78
Spearman correlation for ratings
performed independently
.85, .96, .92

ICC .993 (blinded

rater) ICC .928
(unblinded)

Intrarater Reliability

Cronbach a .93

Cronbach a .89

PABAK .65
PABAK .3
PABAK .91
ICC .927 (inclusion)
ICC .870 (month 12)

Interrater Reliability

Comella et al 2003

Barry et al 1999

Movement Disorders- Battini et al 2009

Childhood Rating
Scale 0-3

Barry-Albright
Dystonia Scale
Unified Dystonia
Rating Scale
Global Dystonia
Rating Scale
Movement DisordersChildhood Rating
Scale

Comella et al 2003
Burke et al 1985

Hypertonia
Jethwa et al 2010
Assessment Tool
Spasticity
Cronbach a 1.0
Dystonia
Cronbach a .79
Rigidity
Not evaluated
Burke-Fahn-Marsden Krystkowiak et al 2007
Movement Scale

Study

Table 1. Internal Consistency, Reliability, Validity, and Clinical Applicability of Clinical Scales.

Pavone et al

343

disability scale is 30. The Burke-Fahn-Marsden Movement

Scale was first validated in a small study consisting of 10
patients (Table 1). In this study, the reliability, interrater agreement, and concurrent validity were assessed.14 It has since been
shown to be reliable in 2 larger, multicenter trials.15,16
The Barry-Albright Dystonia Scale (BAD) was developed to
address the concerns with the Burke-Fahn-Marsden Movement
Scale in evaluating secondary dystonias.17 The Burke-FahnMarsden Movement Scale is predominantly based on function
and was designed to evaluate primary dystonia. Individuals
with secondary dystonia may have cognitive involvement, may
be unable to follow instructions, and have difficulty with activities of daily living. Young children may also have difficulty
following instructions and need assistance with activities of
daily living. The Barry-Albright Dystonia Scale was developed
to quantify the severity of posturing and involuntary movements that develop because of dystonia. The quantification of
the movements allows a clinician to track changes in movement that may ease caregiving or the comfort of the patient, but
not change in function. Two components of the Burke-FahnMarsden Movement Scale are not included in the BarryAlbright Dystonia Scale: provoking factors and disability.17
In addition, the Barry-Albright Dystonia Scale does not weight
different regions. The Barry-Albright Dystonia Scale is a 5point ordinal scale that was designed to assess secondary dystonia in 8 body regions: eyes, mouth, neck, trunk, and the 4
extremities.17 The score in each region is rated 0 (no dystonia)
to 4 (severe dystonia). The maximum scale is 32 and is the sum
of all of the regions measured (Appendix C). The reliability and
responsiveness of the scale was assessed in a small study of
patients with generalized dystonia due to cerebral palsy. The
interrater reliability for the overall scale was found to be excellent, although the reliability of the individual items varied
(Table 1). The interrater reliability improved significantly after
training. The intrarater reliability was found to be good, and
test-retest was acceptable. Overall, when applied by experienced users the Barry-Albright Dystonia Scale is a reliable
clinical tool.
The Unified Dystonia Rating Scale (UDRS) was developed
by the Dystonia Study Group (DSG) in 1997 to create a more
detailed evaluation, as well as address what they perceived
were limitations in the Burke-Fahn-Marsden Movement Scale.
The limitations included variable definition of body areas and a
weighting factor of 0.5 that halves the dystonia involvement in
the eyes, mouth, and neck.15 The Dystonia Study Group also
created a standardized video protocol for assessing dystonia
and the Global Dystonia Rating Scale (GDS). The Unified Dystonia Rating Scale evaluates severity and duration of dystonia
in each body region. The severity rating is from 0 (no dystonia)
to 4 (extreme dystonia). The duration rating is based on a previously validated scale from within the Toronto Spasmodic
Torticollis Scale.15,18-20 Fourteen body regions are assessed:
eyes and upper face, lower face, jaw and tongue, larynx, neck,
trunk, shoulder/proximal arm (right and left), distal arm/hand
(right and left), proximal leg (right and left), and distal leg/foot
(right and left). The maximum score for the Unified Dystonia

Rating Scale is 112. The Unified Dystonia Rating Scale

includes more body regions and is more specific in defining the
region than the Burke-Fahn-Marsden Movement Scale. There
are no weighting factors for any body region in the Unified
Dystonia Rating Scale as in the Burke-Fahn-Marsden Movement Scale. The Unified Dystonia Rating Scale, Burke-FahnMarsden Movement Scale and Global Dystonia Rating Scale
were found to have a high level of internal consistency and
interrater reliability for the total score in assessing primary dystonia in a large multicenter trial by Comella et al15 (Table 1).
The group also examined the subitems of the scales. They
found that the motor severity in the Unified Dystonia Rating
Scale and Burke-Fahn-Marsden Movement Scale had
higher levels of agreement than the duration factor for the
Unified Dystonia Rating Scale or provoking factor for the
Burke-Fahn-Marsden Movement Scale. The interrater agreement was lowest for the larynx and speech for the Unified Dystonia Rating Scale and the Global Dystonia Rating Scale. The
upper face and eyes of the Unified Dystonia Rating Scale and
Burke-Fahn-Marsden Movement Scale demonstrated
the lowest agreement.15
The Global Dystonia Rating Scale rates dystonia in the same
14 body regions as used in the Unified Dystonia Rating Scale.
Each body region is scored from 0 (no dystonia) to 10 (severe
dystonia) and summated for a maximum score of 140. Because
dystonia is continuously changing, for the scoring system the
highest rating that occurs during the observation period is used.
This scale does not include any modifying or weighting factors
as in the Burke-Fahn-Marsden Movement Scale. In a large
multicenter study, the Global Dystonia Rating Scale had a high
level of internal consistency and interrater reliability (Table 1).
The Global Dystonia Rating Scale has also been found to be
very easy to apply and useful in measuring dystonia when compared to the Unified Dystonia Rating Scale and Burke-FahnMarsden Movement Scale.15
As discussed above, the Burke-Fahn-Marsden Movement
Scale and Unified Dystonia Rating Scale were originally
designed to assess primary dystonia, whereas the BarryAlbright Dystonia Scale was developed to assess secondary
dystonia. The reliability and validity of the Burke-FahnMarsden Movement Scale, Unified Dystonia Rating Scale, and
Barry-Albright Dystonia Scale in bilateral dystonic cerebral
palsy was evaluated in a small study of 10 patients.21 The
Barry-Albright Dystonia Scale was found to have good interrater reliability, but poor reliability for measurement of dystonia
in the eyes, mouth, and neck. The Burke-Fahn-Marsden Movement Scale demonstrated moderate reliability for provoking
factors and good reliability for the severity factor and total
score. The Unified Dystonia Rating Scale overall demonstrated
acceptable reliability for the duration and severity but many of
the subitems on both scales had poor reliability.21
Many of the scales used to assess movement disorders were
designed for adults. The Movement Disorder-Childhood Rating Scale was designed to clinically evaluate movement disorders in children and adolescents aged 4 to 18 years.22 The scale
assesses the severity of the movement disorder as well as how it

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Journal of Child Neurology 28(3)

affects motor function and activities of daily living.22 There are

2 components to the Childhood rating scale: the general assessment and the movement severity. The general assessment
includes 4 areas: motor function, oral/verbal function, selfcare, and attention/alertness. The motor function portion evaluates 7 items, including head control, sitting position, standing
position, walking, reaching, grasping, and handwriting. Oral/
verbal function includes swallowing, drooling, and language.
The self-care section includes dressing, feeding, and personal
care. Lastly, the attention and alertness are examined during the
observation period and at home. In assessing the movement
disorder, the severity of the movement is evaluated at rest and
during specific tasks in 7 body regions: eye and periorbital
region, face, tongue and perioral region, neck, trunk, upper
limbs, and lower limbs. The items are scored 0 (no signs) to
4 (most severe findings). Incorporated in the assessment of this
scale is a video protocol. In a study looking at 61 children and
adolescents aged 4 to 18 years with different types of movement disorders the Movement Disorder-Childhood Rating
Scale was found to have high interrater reliability, and high
internal consistency (Table 1).19
The Movement Disorder-Childhood Rating Scale was
revised to develop the Movement Disorder-Childhood Rating
Scale 0-3 to be used in children younger than age 4 years. This
scale is also divided into 2 parts: the general assessment and the
movement severity. The general assessment score is the
adjusted scale and is the sum of 10 items instead of 15. The
items that assess handwriting, language, and 3 items from
self-care were removed. Each item in the general assessment
is scored 0 (no signs) to 4 (most severe). The severity assessment is scored 0 to 2. The reliability and consistency was tested
on 40 children. The scale was found to have high interrater
agreement and a high degree of consistency on several items
(Table 1).23
Dystonia is challenging to measure because it is dynamic and
often mixed with other types of movement disorders. The scales
discussed above have been found to be valid and reliable and can
assist the clinician in identifying what type of movement disorder
is present. Once the movement disorder is identified, the most
appropriate therapeutic regimen can be determined and a scale
may be used to assess the efficacy of therapeutic interventions.
Beyond the clinical utilities, these scales will continue to serve
as targets of and measures for research.

Kinematic Measures
The clinical scales qualitatively measure dystonia, but kinematic measures analyze fine details of movement that are
unable to be seen or differentiated with the human eye. Kinematics involve the motion of a body without considering the
forces that cause the motion. Therefore, kinematics is described
in terms of position such as joint angles and jerk. It is also
described in terms of velocity and acceleration. Muscle activity
and other forces are not considered. Several different measurement techniques are employed to measure kinematics including

digital and infrared-based video systems, electromagnetic systems, and electrogoniometry.24

Kinematic measures are most familiar in the clinical evaluation of children with dystonia in the form of gait analyses. In
assessing gait, for example, one approach is to use spherical
retroreflective markers attached to the skin surface at key anatomic landmarks20 mm superior to the greater trochanter,
the lateral femoral condyle, the lateral malleolus, and the fifth
metatarsal joint.25,26 A multi-camera video-based system
can then be used to collect the kinematic data. There are 2dimensional models and then there are more complex 3dimensional gait analyses that provide a new plane and a host
of additional measurements. Pelvic rotation and knee flexion
during stance are just 2 examples of the numerous measures
available; a full listing is beyond the scope of this review. It is
important to note that age is a factor in gait analyses in that childrens data are more variable than adult data.27,28 Still, gait analyses are often used as the measurement tool in assessing change
after interventions such as botulinum toxin injections, dorsal rhizotomy, or other surgical procedures.29-31 A few studies have
explored dystonia in children using some of these kinematic
measures.
Gait analysis was used in a study that examined the biomechanic characteristics of knee joint motion and walking in children and adults with cerebral palsy in order to differentiate
spasticity and dystonia.5 The minimum knee angle during
stance phase was found to be smaller in patients with spasticity
compared to dystonia, knee range of motion in stance phase
was smaller in subjects with dystonia compared to spasticity,
and the self-selected walking velocity was lower in subjects
with dystonia compared to spasticity. The slower walking in
subjects with dystonia appeared to be related to a reduction
in limb strength.
Kinematic measures, though more often explored clinically
in the lower limbs during gait analysis, also are studied for
upper extremity use. Retroreflective markers as used in gait
analysis can also be used for upper extremity tasks, but the
approaches are more variable. The large degrees of freedom
at the shoulder and the skin movement that occurs during forearm and scapular motion make set up and analysis of upper
extremity kinematic data more difficult.29,32,33 Another
approach is to use marker clusters at the segmental center of
mass and to calibrate bony landmarks with reference to the
marker clusters, but this approach limits the ability to assess
joint angles.33,34 However, the difficulty in defining a repeatable task and the difficulty in establishing a representative mathematical model combined with the lack of commercial
software hinders the clinical use of 3-dimensional upper limb
kinematics.29 One study compared children with dyskinetic
cerebral palsy (all had dystonia) to controls in terms of arm trajectory during an unconstrained outward-reaching movement.8
The children with dystonia had an increased variability and a
lack of straight-line trajectories compared with controls.8 This
finding is consistent with the hypothesis that an inability to
remove unwanted and variable components of movement
might underlie the movement disorder as opposed to the

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345

movement disorder being due to inappropriate selection or

planning of an incorrect trajectory or due to fixed dystonic postures.8 In another study, it was found that children with dystonia also demonstrated decreased speed, greater variability, and
pauses at targets compared with controls during an upper limb
reaching task.4 The decreased speed was mostly due to difficulty in reversing reaching direction, and increased variability
was associated with large fluctuations in the duration of the
pauses at targets rather than due to variations in velocities. Both
of these findings could be related to difficulty with agonist/
antagonist muscle activation when reversing directions; this
would fit with the evidence suggesting basal ganglia involvement. The children with dystonia showed lower levels of cocontraction compared with controls and that level of co-contraction did not correlate with the severity of the dystonia.
Other abnormalities affecting motion (weakness, spasticity,
bradykinesia, choreoathetosis) may have contributed to their
findings. In a study comparing children with movement disorders to children with hemiplegic cerebral palsy and controls in
a reach-to-grasp task, both children with movement disorders
and hemiplegic cerebral palsy showed increased movement
duration.35 Children with movement disorders further showed
increased head and trunk movements in all 3 planesfrontal,
transverse, and sagittal. Examination of temporal-spatial parameters during a reach-and-grasp cycle in children with hemiplegic cerebral palsy compared to controls validate that children
with dyskinetic cerebral palsy had slower movement.36
Targeting and size of target changes the speed of movement
for children with dystonia. In a study involving a task of reaching to target buttons of different sizes, children with dystonia
moved slower than controls at all button sizes and their speed
was more sensitive to changes in button size.37 This finding
implies that children with dystonia require larger targets to
achieve speed comparable to controls.37 Functional implication
for this slow movement and target size is demonstrated with
control during computer use in adolescents with cerebral
palsy.38 It was found that cursor movement time was significantly slower when the target was smaller.
Given that several studies have found kinematic differences
between children with dystonia and controls, a few studies have
examined kinematic measures within groups of children with
dystonia. Children with more severe dystonia have a more
curved path during a reaching task compared to children with
less severe dystonia.39 This finding has been reproduced, when
comparing Manual Ability Classification System scores.
Movement times were longer for those children with dyskinetic
cerebral palsy with higher Manual Ability Classification System scores.36 Paralleling this finding, another study found
slower cursor movement times for adolescents with cerebral
palsy with higher Manual Ability Classification System scores
during a computer cursor control task.38

Implications for Function

Dystonia represents an impairment, which interferes with
activities and the childs ability to participate in his or her

biosocial role. The main goal of rehabilitation is to improve

function and to improve the activity and participation arenas
of the WHO-ICF. To maximize function, a multidisciplinary
team approach is necessary. Reducing the severity of dystonia
will not always change function, because most function combines
cognitive, motivation, sensory processing and motor ability.
Dystonia is generalized or focal, and action induced or posture induced. Because movement is disrupted by intermittent
torsional hypertonia, it creates problems with gradation of
movement, accuracy, speed, and positioning of the limb or
body in space for both fine and gross motor movements. Dystonia may be unpredictable and can have varying severity. Dystonia may challenge all areas of function.
Functional domains in rehabilitation include mobility, selfcare or activities of daily living, communication, and cognition.
Many functional measures exist that have allowed numerical
ratings of function. A comprehensive yet minimal database
of 18 items comprises the Functional Independence Measure
and allows for comparison of functional gain for individuals,
programs, and centers. The Wee Functional Independence
Measure is the downward extension for ages 3 to 12 years if
cognitively normal and can be utilized for individuals cognitively delayed.40 Other functional measures for children
include the Pediatric Evaluation of Disability Inventory
(PEDI),41 which accounts for rehabilitation interventions such
as wheelchairs or augmentative communication devices. The
problem with the broad stroke measures such as the Functional
Independence Measure is the lack of sensitivity to change in
individuals. The Burke-Fahn-Marsden Movement Scale rating
scale combines the ideas of impairment and disability ratings
though does not take into account the developmental aspects
of childhood.
For children, the overlapping process of development may
confound issues of improvement. It is critical to utilize both
types of measures for children with dystonia that manifest early
in infancy and childhood. The developmental tests have criterion norms and reference ages. Understanding the skills set of a
child combines both knowledge of development and evolving
functional independence. The earliest functional and developmental skills to evolve are feeding and mobility. Some milestones are predictive of these later tasks. For example, the
child with cerebral palsy who has the ability to sit independently
will generally have a good prognosis for later ambulation.
Identifying the functional issues that dystonia interferes
with will allow the use of compensatory treatment or other therapeutic equipment. In addition, there is good reason to utilize
treatments either medically or within our rehabilitation armamentarium to improve function versus just decreasing the
impairment. Frequently medication management is evaluated
by improvement in function.
A good example of basic rehabilitative management versus
the medical management is the varus-supinated foot, commonly observed with lower limb dystonia. The foot, which
will very probably respond to botulinum toxin injections to the
posterior tibialis muscle temporarily, certainly will need a stabilizing orthotic regardless of medication management to

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Journal of Child Neurology 28(3)

ensure foot stability and prepositioning for walking. Mobility

assistance is frequently necessary because of poor balance or
uncontrolled, unpredictable movements. Walkers and canes
allow ambulation despite insufficient stability for stance.
Regardless of severity of the impairment, technology is now
available that can make a difference in function. Of all the
functional skills, communication is held to be the highest priority for the adult with disabilities.42 Computer interfaces harness
the capabilities of those who are unable to execute fine motor
or oral motor tasks for communications and integration into
school and future employment.

Conclusion
Evaluating children with dystonia is difficult because a spectrum of abnormalities may be present. Although dystonia may
be the dominant manifestation of a childs disease or condition,
several other abnormalities affecting motion must be considered,
including weakness, spasticity, impaired selective motor control,
bradykinesia, choreoathetosis, ataxia, and sensory impairments.
Recognition that dystonia interferes with function once
spasticity is reduced has led clinicians to further appreciate and

discriminate and rate dystonia. Many valid and reliable clinical

scales are available, and we advocate using a scale that commits to a severity rating. The scales are important to employ
in clinical and research settings.
The evaluation of movement and dystonia in particular has
improved with technology, high-speed cameras, and kinematics. Kinematics in dystonia differs from spasticity and can
be investigated during functional tasks such as gait or reach.
Despite the relatively small amount of literature, the kinematic evidence points to decreased speed and/or increased
movement variability in children with dystonia. It follows that
kinematic measures appear to have a role in analyzing children
with dystonia in both the clinical and the research realms. As
virtual reality and wireless kinematics evolve, this proves to
be promising for discriminating hypertonia, evaluating severity, and treatment.
This review has given an overview for those clinical
tools available. The scales can be put into practice for the
day-to-day management of patients. Importantly, the kinematics provides new insight and understanding into the
mechanisms of dystonia and how they impact motor control
and function.

Appendix A: Hypertonia Assessment Tool

Type of
Hypertonia

Hypertonia Assessment Tool Item

Scoring Guidelines

1. Increased involuntary movements/

postures of the designated limb with
tactile stimulus of a distal body part
2. Increased involuntary movements/
postures with purposeful movements
of a distal body part
3. Velocity-dependent resistance to
stretch

0 No involuntary movements or postures observed

1 Involuntary movements or postures observed

Dystonia

0 No involuntary movements or postures observed

1 Involuntary movements or postures observed

Dystonia

4. Presence of a spastic catch

5. Equal resistance to passive stretch
during bidirectional movement of a
joint
6. Increased tone with movement of a
distal body part
7. Maintenance of a limb position after
passive movement

0 No increased resistance noticed during fast stretch compared to slow

Spasticity
stretch
1 Increased resistance noticed during fast stretch compared to slow stretch
0 No spastic catch noted
Spasticity
1 Spastic catch noted
0 Equal resistance not noted with bidirectional movement
Rigidity
1 Equal resistance noted with bidirectional movement
0
1
0
1

No increased tone noted with purposeful movement

Greater tone noted with purposeful movement
Limb returns (partially or fully) to original position
Limb remains in final position of stretch

Dystonia
Rigidity

Source: Adapted from Jethwa A, Mink J, Macarthur C, et al. Development of the Hypertonia Assessment Tool (HAT): a discriminative tool for hypertonia in
children. Dev Med Child Neurol. 2010;52(5): e83-e77.

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347
3.

Appendix B: Burke-Fahn-Marsden
Movement Scale
MOVEMENT SCALE
Provoking
Factor
Eyes
Mouth
Speech/swallow
Neck
Right. arm
Left. arm
Trunk
Right. leg
Left. leg











0-4
0-4
0-4
0-4
0-4
0-4
0-4
0-4
0-4

Severity
Factor

Weight

Product

0-4
0-4
0-4
0-4
0-4
0-4
0-4
0-4
0-4

0.5
0.5
1.0
0.5
1.0
1.0
1.0
1.0
1.0

0-8
0-8
0-16
0-8
0-16
0-16
0-16
0-16
0-16

Sum:
(maximum 120)
I. Provoking Factors
A. General
0.
1.
2.
3.

No dystonia at rest or with action

Dystonia on a particular action
Dystonia on many actions
Dystonia on action of distant
part of body or intermittently
at rest
4. Dystonia present at rest
B. Speech and Swallow
0. Occasional, either or both
1. Frequent either
2. Frequent one and occasional
other
3. Frequent both
II. Severity Factors
Eyes
0. No dystonia present
1. Slight. Occasional blinking
2. Mild. Frequent blinking
without prolonged spasms of
eye closure
3. Moderate. Prolonged spasms of
eyelid closure, but eyes open
most of time
4. Severe. Prolonged spasms of
eyelid closure, with eyes closed
30% of the time
Mouth
0. No dystonia present
1. Slight. Occasional
grimacing or other
mouth movements (eg, jaw open
or clenched; tongue movement)
2. Mild. Movement present less
than 50% of the time

Moderate dystonic movements

or contractions present most of
the time
4. Severe dystonic movements or
contractions present most of
time
Speech and swallowing
0. Normal
1. Slightly involved; speech
easily understood or occasional
choking
2. Some difficulty in understanding speech or frequent choking
3. Marked difficulty in understanding speech or inability to
swallow firm foods
4. Complete or almost complete
anarthria, or marked difficulty
in swallowing soft foods and
liquids
Neck
0. No dystonia present
1. Slight. Occasional pulling
2. Obvious torticollis, but mild
3. Moderate pulling
4. Extreme pulling
Arm
0. No dystonia present
1. Slight dystonia. Clinically
insignificant
2. Mild. Obvious dystonia, but not
disabling
3. Moderate. Able to grasp,
with some manual function
4. Severe. No useful grasp
Trunk
0. No dystonia present
1. Slight bending, clinically
insignificant
2. Definite bending, but not
interfering with standing or
walking
3. Moderate bending; interfering
with standing or walking
4. Extreme bending of trunk
preventing standing or walking
Leg
0. No dystonia present
1. Slight dystonia, but not causing
impairment
2. Mild dystonia. Walks briskly
and unaided
3. Moderate dystonia. Severely
impairs walking or requires
assistance

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Journal of Child Neurology 28(3)

4.

Severe. Unable to stand or walk

on involved

DISABILITY SCALE

Appendix C: Barry Albright Dystonia Scale

Eyes: Signs of dystonia of the eyes include: Prolonged eyelid
spasms and/or forced eye deviations
Function

Score (Sum of items below)

Speech

0
1
2
3
4
0
1
2
3
4
0
1
2
3
4
0
1
2

Handwriting (tremor
or dystonia)

Feeding

Eating/Swallowing

3
4
Hygiene

Dressing

Walking

0
1
2
3
4
0
1
2
3
4
0
1
2
3
4
5

Normal
Slight involved; easily understood
Some difficulty in understanding
Marked difficulty in understanding
Complete or almost complete anarthria
Normal
Slight difficulty; legible
Almost illegible
Illegible
Unable to grasp to maintain hold on pen
Normal
Uses tricks; independent
Can feed, but not cut
Finger food only
Completely dependent
Normal
Occasional choking
Chokes frequently; difficulty
swallowing
Unable to swallow firm foods
Marked difficulty swallowing soft foods
and liquids
Normal
Clumsy, independent
Needs help with some activities
Needs help with most activities
Needs help with all activities
Normal
Clumsy, independent
Needs help with some activities
Needs help with most activities
Helpless
Normal
Slightly abnormal; hardly noticeable
Moderately abnormal; obvious to nave
observer
Considerably abnormal
Needs assistance to walk
Wheelchair-bound

Source: Adapted from Burke R, Fhan S, Marsden CD, et al. Validity and
reliability of a rating scale for primary torsion dystonias. Neurology.
1985;35(1):73-77.

0. Absence of eye dystonia

1. Slight. Dystonia less than 10% of the time
2. Mild. Frequent blinking without prolonged spasms
of eye closure and/or eye movements less than 50%
of the time

3. Moderate. Prolonged spasms of eyelid closure, but

eyes open most of the time
4. Severe. Prolonged Spasms of eyelid closure, with eyes
closed at least 30% of the time
Mouth: Signs of dystonia of mouth include: Grimacing,
clenched or deviated jaw, forced open mouth, and/or forceful
tongue thrusting
0. Absence of mouth dystonia
1. Slight. Dystonia less than 10% of the time and does not
interfere with speech or feeding
2. Mild. Dystonia less than 50% of the time and does not
interfere with speech or feeding
3. Moderate. Dystonia more than 50% of the time, or
dystonia that interferes with speech or feeding
4. Severe. Dystonia more than 50% of the time or dystonia that prevents speech or feeding
Neck: Signs of dystonia of the neck include: Pulling of the neck
into any plane of motion: Extension, flexion, lateral flexion, or
rotation
0. Absence of neck dystonia
1. Slight. Pulling less than 10% of the time and does not
interfere with lying, sitting, standing or walking
2. Mild. Pulling less than 50% of the time and does not
interfere with lying, sitting, standing, or walking
3. Moderate. Pulling more than 50% of the time or dystonia
that interferes with lying, sitting, standing, or walking
4. Severe. Pulling more than 50% of the time or dystonia
that prevents sitting in standard wheelchair, standing
or, walking (i e, requires more than standard head rest
for seating)
Trunk:
0. Absence of trunk dystonia
1. Slight. Pulling less than 10% of the time and does not
interfere with lying, sitting, standing, or walking
2. Mild. Pulling less than 50% of the time and does not
interfere with lying, sitting, standing, or walking
3. Moderate. Pulling more than 50% of the time or dystonia that interferes with lying, sitting, standing, or
walking
4. Severe. Pulling more than 50% of the time or dystonia
that prevents sitting in standard wheelchair, standing or
walking
Upper Extremities (Left/Right):
0. Absence of upper extremity dystonia
1. Slight. Dystonia less than 10% of the time and does not
interfere with normal positioning or functional activities
2. Mild. Dystonia less than 50% of the time and does not
interfere with normal positioning or functional
activities
3. Moderate. Dystonia more than 50% of the time, or
dystonia that interferes with normal positioning or
functional activities
4. Severe. Dystonia more than 50% of the time, or dystonia that prevents normal positioning or upper extremity function; i e, arms restrained in wheelchair to
prevent injury

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Lower Extremities (Left/Right):

0. Absence of lower extremity dystonia
1. Slight. Dystonia less than 10% of the time and does not
interfere with normal positioning or functional
activities
2. Mild. Dystonia less than 50% of the time and does not
interfere with normal positioning or functional
activities
3. Moderate. Dystonia more than 50% of the time, or
dystonia that interferes with normal positioning or
lower extremity weight bearing or function
4. Severe. Dystonia more than 50% of the time, or dystonia that prevents normal positioning or lower extremity weight bearing or function
Source: Adapted from Barry M, VanSearingen J, Albright AL.
Reliability and responsiveness of the Barry-Albright-Dystonia
Scale. Dev Med Child Neurol. 1999;41(6):404-411.
Author Contributions
LP and JB completed the literature review. LP wrote the section on
clinical measures, completed the figures and tables, and formatted the
article. JB wrote the section on kinematic measures and a majority of
the definition and etiology section. DG-S conceptualized the review
and provided supervision throughout the writing and editing process.
DG-S also completed the introduction, the section on implication of
function, and the conclusion. All 3 authors reviewed and edited the
article.

Declaration of Conflicting Interests

The authors declared the following potential conflicts of interest with
respect to the research, authorship, and/or publication of this article:
Deborah Gaebler-Spira, MD, is a consultant for Merz and Rehab Tech.

Funding
The authors received no financial support for the research, authorship,
and/or publication of this article.

Ethical Approval
This article is a review of the literature and therefore did not require
review by the institutional review board/ethics committee.

References
1. Jethwa A, Mink J, Macarthur C, et al. Development of the Hypertonia Assessment Tool (HAT): a discriminative tool for hypertonia in children. Dev Med Child Neurol. 2010;52:e83-e87.
2. World Health Organization. International classification of
functioning, disability and health. https://www.who.int/classifica
tions/icf/training/icfbeginnersguide.pdf. Accessed July 18, 2011.
3. Sanger T, Delgado M, Gaebler-Spira D, et al. Classification and
definitions of disorders causing hypertonia in childhood. Pediatrics. 2003;111:e89-e97.
4. Malfait N, Sanger T. Does dystonia always include co-contraction? A study of unconstrained reaching in children with primary
and secondary dystonia. Exp Brain Res. 2007;176:206-216.

5. Lebiedowska M, Gaebler-Spira D, Burns R, Fisk J. Biomechanic

characteristics of patients with spastic and dystonic hypertonia in
cerebral palsy. Arch Phys Med Rehabil. 2004;85:875-880.
6. Hallet M. Physiology of dystonia. In: Fahn S, Marsden CD,
DeLong M, eds. Advances in Neurology: Vol 78. Philadelphia,
PA: Lippincott-Raven; 1998:11-25.
7. Sanger T, Chen D, Fehlings D, et al. Definition and classification
of hyperkinetic movements in childhood. Mov Disord. 2010;25:
1538-1549.
8. Sanger T. Arm trajectories in dyskinetic cerebral palsy have
increased random variability. J Child Neurol. 2006;21:551-557.
9. Ikeda A, Shibasaki H, Kaji R, et al. Abnormal sensorimotor integration in writers cramp: study of contingent negative variation.
Mov Disord 1996;11:683-90.
10. Kaji R, Ikeda A, Ikeda T, et al. Physiological study of cervical
dystonia. Task-specific abnormality in contingent negative variation. Brain 1995;118:511-522.
11. Yazawa S, Ikeda A, Kaji, et al. Abnormal cortical processing of
voluntary muscle relaxation in patients with focal hand dystonia
studied by movement-related potentials. Brain. 1999;122:13571366.
12. Odergren T, Iwasaki N, Borg J, et al. Impaired sensory-motor
integration during grasping in writers cramp. Brain 1996;119:
569-583.
13. Fehlings D, Switzer L, Jethwa A, et al. Hypertonia Assessment
Tool (HAT): User Manual. 2011. Available from http://www.hollandbloorview.ca/research/scientistprofiles/documents/HATUser
Manual_Nov20102.pdf.
14. Burke R, Fhan S, Marsden CD, et al. Validity and reliability of
a rating scale for primary torsion dystonias. Neurology. 1985;
35:73-77.
15. Comella C, Leurgan S, Wuu J, et al. Rating scales for dystonia:
A multicenter assessment. Mov Disord. 2003;18:303-312.
16. Krystkowiak P, Tezenas du Montcel S, Vercueil L, et al. Reliability of the Burke-Fahn-Marsden Scale in a multicenter trial for
dystonia. Mov Disord. 2007;22(5):685-689.
17. Barry M, VanSearingen J, Albright AL. Reliability and responsiveness of the Barry-Albright Dystonia Scale. Dev Med Child
Neurol. 1999;41:404-411.
18. Consky ES, Basinki A, Belle L, et al. The Toronto Western
Spasmodic Torticollis Rating Scale (TWSTRS): assessment of
the validity and inter-rater reliability. Neurology. 1990;40(suppl):
445.
19. Consky ES, Lang AE. Clinical assessments of patients with
cervical dystonia. In: Jankovic J, Hallet M, eds. Therapy With
Botulinum Toxin. New York, NY: Marcel Dekker, Inc; 1994:
211-237.
20. Comella CL, Stebbins GT, Goetz CG, et al. Teaching tape for the
motor section of the Toronto Western Spasmodic Torticollis
Scale. Mov Disord 1997;12:570-575.
21. Monbaliu E, Ortibus E, Roelens F, et al. Ratings for dystonia in
cerebral palsy: reliability and validity. Dev Med Child Neurol.
2010;52:570-575.
22. Battini R, Sgandurra G, Petacchi E, et al. Movement DisorderChildhood Rating Scale: reliability and validity. Pediatr Neurol.
2008;39:259-265.

Downloaded from jcn.sagepub.com at Scientific library of Moscow State University on January 7, 2014

350

Journal of Child Neurology 28(3)

23. Battini R, Guzzetta A, Sgandurra G, et al. Scale for evaluation of

movement disorders in the first three years of life. Pediatr Neurol.
2009;40:258-265.
24. Dayanidhi S. Kinematics: overview & techniques. Paper presented
at the Taskforce on Childhood Motor Disorders; July 12, 2009;
Texas Scottish Rite Hospital, Dallas, TX.
25. Hallet M, Lebiedowska M, Thomas S, et al. Locomotion of
autistic adults. Arch Neurol. 1993;50:1304-1308.
26. Lebiedowska M. The kinematic consequences of invariant
dynamics in children 6-18 years of age. J Biomech. 2008;41:
2458-2464.
27. Stolz H, Kuhtz-Buschbeck J, Mondwurf C, et al. Retest reliability
of spatiotemporal gait parameters in children and adults. Gait
Posture. 1998;7:125-130.
28. McGinley J, Baker R, Wolfe R, Morria M. The reliability of threedimensional kinematic gait measurements: a systematic review.
Gait Posture. 2009;29:360-369.
29. Mackey A, Walt S, Stott S. Deficits in upper-limb task performance
in children with hemiplegic cerebral palsy as defined by 3dimensional kinematics. Arch Phys Med Rehabil. 2006;87:207-215.
30. Schwartz M, Vieweger E, Stout J, et al. Comprehensive treatment
of ambulatory children with cerebral palsy: an outcome assessment. J Pediatr Orthop. 2004;24:45-53.
31. Sutherland D, Kaufman K, Wyatt M, et al. Double-blind study of
botulinum A toxin injections into the gastrocnemius muscle in
patients with cerebral palsy. Gait Posture. 1999;10:1-9.
32. Anglin C, Wyss UP. Review of arm motion analyses. Proc Inst
Mech Eng H. 2000;214:541-555.
33. Jaspers E, Desloovere K, Bruynicnkx H, et al. Review of quantitative measurements of upper limb movements in hemiplegic cerebral palsy. Gait Posture. 2009;30:395-404.

34. Wu G, Cavanagh PR. ISB recommendations for standardization in the reporting of kinematic data. J Biomech. 1995;28:
1257-1261.
35. Coluccini M, Maini E, Martelloni C, et al. Kinematic characterization of functional reach to grasp in normal and in motor disabled children. Gait Posture. 2007;25:493-501.
36. Butler E, Ladd A, LaMont L, Rose J. Temporal-spatial parameters
of the upper limb during a reach & grasp cycle for children. Gait
Posture 2010;32:301-306.
37. Sanger T, Kaiser J, Placek B. Reaching movements in childhood
dystonia contain signal-dependent noise. J Child Neurol 2005;20:
489-496.
38. Davies T, Chau T, Fehlings D, et al. How is cursor control during
computer use by adolescents with cerebral palsy related to their
MACS level? In: Proceedings of the American Academy for Cerebral Palsy and Developmental Medicine; September 22-25,
2010; Washington, DC.
39. Gordon L, Keller J, Stashinko E, et al. Can Spasticity and dystonia
be independently measured in cerebral palsy? Pediatr Neurol.
2006;35:375-381.
40. Msall M, DiGaudio K, Rogers B, et al. The functional independence measure for children (WeeFim): conceptual basis and pilot
use in children with developmental disabilities. Clin Pediatr.
1994;33:431-438.
41. Haley SM, Coster WJ, Ludlow LH, et al. Pediatric Evaluation of
Disability Inventory (PEDI): Development Standardization and
Administration Manual, Version 1.0. Boston, New England Medical Center, 1992.
42. Bleck E. Goals, treatment, and management, In: Orthopedic Management of Cerebral Palsy. Philadelphia, PA: JB Lippincott;
1987:142-143.

Downloaded from jcn.sagepub.com at Scientific library of Moscow State University on January 7, 2014