Pediatric movement disorders

Terence D. Sanger

Purpose of review Introduction

Pediatric movement disorders are a heterogeneous group of Adult movement disorders frequently share terminology
symptoms that occur in the context of a large number of with the symptoms of those disorders, so that ‘parkin-
different neurological diseases. Accurate diagnosis and sonism’ occurs in Parkinson’s disease, ‘dystonia’ occurs
quantification of these disorders is essential for determining in primary torsion dystonia, ‘chorea’ occurs in Hunting-
outcome, appropriate treatment, and criteria for inclusion in ton’s chorea, and so forth. In children, movement
research trials. The purpose of this review is to summarize disorders are more commonly symptoms of other
recent advances in diagnosis and treatment for childhood diseases. For example, dystonia may be the secondary
movement disorders. result of any of a large number of static or progressive
Recent findings diseases, and categorization of the type of symptom is
The ultimate goal is to discover new treatments that can lead to only one of many steps needed to determine an
measurable improvement in functional outcome for affected etiologic diagnosis. Tic disorders are an important
children. In order to accomplish this goal, we must have exception, but we will not discuss tic disorders further
consistent definitions and accurate measurements to determine in this review.
the diagnosis and severity for each child in a clinic or research
trial. Recent progress in defining childhood movement disorders Childhood movement disorders frequently involve
has led to consensus definitions of different types of hypertonia. dystonia or spasticity. ‘Negative symptoms’ (defined
There has also been progress in the development of outcome below, and including weakness, ataxia, and poorly
measures that relate to meaningful functional performance in a differentiated control) are also an extremely frequent
variety of skill areas. Most exciting is the prospect of new contributor to disability. The most common cause of
treatments, and we survey the current non-medical, medical, movement disorders in children is cerebral palsy, but this
and surgical therapies for childhood motor disorders. disease should be understood as a very heterogeneous
Summary group of disorders caused by static injury to one or more
Although pediatric movement disorders are a complex and often motor systems of the brain. A variety of genetic or
poorly understood group of symptoms, recent work has shown metabolic diseases in childhood can present with move-
that there is a possibility of defining, measuring, and ultimately ment disorders such as dystonia or parkinsonism [1–7].
treating these debilitating diseases. The similarity of symptoms attributable to different
neurological diseases suggests that, whatever the me-
Keywords chanism of injury, damage to motor systems of the brain
movement disorders, dystonia, spasticity, rigidity, myoclonus, is reflected by a ‘final common pathway’ of motor
ataxia, tremor, chorea, athetosis, pediatric, childhood, disorders for which there is only a limited number of
treatment, diagnosis different characteristic deficits. The nature of the
symptoms may be more determined by the anatomical
Curr Opin Neurol 16:529–535. # 2003 Lippincott Williams & Wilkins. location of injury than by the precise mechanism.

Department of Neurology and Neurological Sciences, Pediatric Movement Disorders The reason for the difference in epidemiology between
Clinic, Stanford University Medical Center, Stanford, California, USA
child and adult disorders is likely to be due to the
Correspondence to Terence D. Sanger, Department of Neurology and Neurological different effects of injury on the developing compared
Sciences, Pediatric Movement Disorders Clinic, Stanford University Medical Center, with the adult brain. For example, hypoxic injury in the
300 Pasteur Drive, A345, Stanford, CA 94305-5235, USA
e-mail: sanger@[Link] prenatal period can have very different effects from
later hypoxic injury [8 . .]. As another example, de-
Current Opinion in Neurology 2003, 16:529–535
creased dopamine in children and young adults appears
# 2003 Lippincott Williams & Wilkins
to be able to cause either dystonia or parkinsonism
1350-7540 [9 .,10–17], whereas later in life primarily parkinsonism
occurs [18] with dystonia in parkinsonian adults existing
primarily as a symptom related to treatment with
dopaminergic medicines. An unchanging injury in
childhood can present with variable or progressive
symptoms due to effects of growth and development
[19], and the effect of a fixed injury may change over
time [14,15,20,21].
DOI: 10.1097/[Link].0000084233.82329.0e 529
530 Movement disorders

The lack of effective disease-specific treatments and the determine the effect of intervention and measure
similarity of symptoms caused by different childhood functional outcome [19].
diseases has led to a search for therapies that may be
symptom-specific rather than disease-specific. This effort Although not usually listed along with other movement
has been hampered by a lack of consistent definitions of disorders, the negative symptoms frequently present in
symptoms and a lack of quantifiable measures of the childhood motor disease may be a more significant
severity of symptoms. It has also been difficult to contributor to disability than the ‘positive symptoms’.
determine the extent to which any given symptom Negative symptoms are usually taken to indicate the
contributes to a child’s disability. In the subsequent lack of a particular function, rather than the addition of
sections, we will discuss the relationship between an excess or uncontrolled movement. In many situations,
specific impairments and disability, and we will review the distinction is difficult. Typical examples of negative
recent developments in definition, quantification, and symptoms include weakness, ataxia, apraxia, and poor
treatment of childhood motor disorders. differentiation of individual movements (this last cate-
gory may be better classified as an apraxia in some
Functional goals cases).
In the absence of effective and risk-free treatments for
many of the diseases that cause movement disorders in Types of childhood movement disorders
children, it becomes essential to categorize and prioritize Classically, the movement disorders have been consid-
goals for functional improvement [19]. The National ered to include ataxia, athetosis, chorea, dystonia,
Center for Medical Rehabilitation Research (NCMRR) myoclonus, bradykinesia, tics, and tremor (dystonia is
has recommended the use of a five-axis scale, in which recognized to have many different manifestations,
pathophysiology (the disease process), impairment (clin- including dystonic spasms, dystonic tremor, repetitive
ical signs and symptoms), functional limitation (specific movements, abnormal fixed postures, and hypertonia,
limitations of movement), disability (limitations in and it may well be that athetosis in children is a
particular tasks), and societal participation (ability to particular expression of dystonia). Although this termi-
participate in age-appropriate activities) are included as nology is prevalent in the adult movement disorders
separate elements describing the impact of disease literature, for children it is important to include the
[22,23]. This classification reminds us that treatment at negative symptoms as well. It is equally important to
one level does not necessarily lead to improvement at consider the role of spasticity, both as a diagnostic
other levels. For example, reduction of spasticity (an finding and as a potential cause of disability. It may be
impairment) does not necessarily lead to improvement in helpful to use the term ‘motor disorder’ to indicate any
an abnormal gait (a functional limitation) although it may impairment of movement, whether including positive or
contribute to ease of transfers to or from a wheelchair (a negative symptoms, and not limited to the ‘movement
disability) and ultimately to greater ability to get to and disorders’ that are more familiar to the adult neurology
from school (societal participation). community.

Selection of appropriate functional interventions for Research in human motor control suggests that voluntary
childhood movement disorders is complicated by the movement can be divided into a planning phase and an
observation that children frequently have more than one execution phase [25–28]. Therefore, when considering
type of symptom as a result of their underlying disease childhood movement disorders, it may be helpful to
[24]. Determining the relationship between particular consider whether a particular disorder is more likely to
symptoms and the child’s functional goals becomes relate to planning (e.g. apraxia) or execution (e.g.
important for determining which symptom to approach weakness). We must also consider disorders for which
first as a target for treatment. there is excessive movement that is not triggered by
volition (e.g. myoclonus). Finally, we must consider
The mixed symptoms in children also complicate the those disorders that are readily apparent to a clinician but
selection of subjects for clinical research trials. By far the which do not necessarily interfere with voluntary move-
largest single group of children is the group with cerebral ment (e.g. spasticity).
palsy. However, the variability of symptom type and
severity in this group makes it very difficult to obtain a Thus in some cases it may be helpful to separate
homogeneous sample, and research studies are often childhood movement disorders into four primary cate-
forced to confine subjects to a subset (such as hemiplegic gories: (1) disorders of tone, (2) disorders of inhibition of
cerebral palsy) or a related disorder with ‘pure’ forms of movement, (3) disorders of execution of movement or
particular symptoms (such as hereditary spastic para- posture, and (4) disorders of movement planning.
plegia). No matter what the difficulties, appropriate Disorders of tone are signs observed by the clinician
categorization of children is required in order to during a neurological examination. These disorders do
 Pediatric movement disorders Sanger 531

not describe the results of voluntary movement, and multiple symptoms including weakness. Disorders of
they may be more important as diagnostic tools than as sensation can lead to many different abnormalities of
determinants of disability. Disorders of inhibition are planning or execution [34], and whether these should be
disorders in which movement is initiated without the considered separately should be investigated [35–37].
child’s intent. The result is extra movement that occurs
either at rest or associated with movement, but which is Advances in defining childhood movement
not closely linked to the particular movement being disorders
performed. Disorders of execution are disorders in which One of the most salient diagnostic features of many
the pattern or magnitude of muscle activation is not childhood motor disorders is the presence of increased
appropriate for the intended task. This will lead to a tone. There has been disagreement between practi-
movement which does not correspond to the child’s tioners of different fields concerning the appropriate
plan. Disorders of planning are disorders in which the labeling of different types of childhood hypertonia. A US
muscle activations are appropriate for the intended National Institutes of Health-sponsored taskforce re-
movement plan, but the movement does not accomplish cently proposed a set of consensus definitions for
the desired task. Under this taxonomy, it may not always spasticity, dystonia, and rigidity as they apply to child-
be possible to assign a particular disorder to a single hood hypertonia [19]:
category. For example, ataxia is likely to be a disorder
both of execution and planning. Dystonia may cause an ‘Spasticity is defined as hypertonia in which one or both
abnormality of tone, but it also affects movement of the following signs are present: (1) resistance to
execution, and hypertonic dystonia could be due to a externally imposed movement increases with increasing
lack of inhibition of antagonist muscle activity. In young speed of stretch and varies with the direction of joint
children or in children with cognitive deficits it may be movement; (2) resistance to externally imposed move-
difficult to distinguish between disorders of planning ment rises rapidly above a threshold speed or joint angle.’
and execution. To illustrate the categories, in Table 1
we have attempted to place examples of specific ‘Dystonia is defined as a movement disorder in which
disorders within the category that best describes the involuntary sustained or intermittent muscle contractions
effects of the disorder. cause twisting and repetitive movements, abnormal
postures, or both.’
The last category in Table 1, disorders of planning, is
perhaps the least well-understood of the childhood ‘Rigidity is defined as hypertonia in which all of the
motor disorders. It is not known whether the adult following are true: (1) the resistance to externally
classification of apraxia (ideational, ideomotor, and limb- imposed joint movement is present at very low speeds
kinetic [29,30]) applies to children. It is not known of movement, does not depend on imposed speed, and
whether speech apraxia [31–33] is related to apraxia does not exhibit a speed or angle threshold; (2)
involving arm or hand movements. Furthermore, it is not simultaneous co-contraction of agonists and antagonists
known whether developmental coordination disorder may occur, and this is reflected in an immediate
[34] is a separate entity from apraxia. Finally, it is not resistance to a reversal of the direction of movement
known whether poorly differentiated control is due to about a joint; (3) the limb does not tend to return toward
apraxia, ataxia, dystonia, or possibly a combination of a particular fixed posture or extreme joint angle; (4)
voluntary activity in distant muscle groups does not lead
to involuntary movements about the rigid joints,
Table 1. Categorization of specific childhood movement disorders
although rigidity may worsen.’
Category Disorder
Disorders of tone Spasticity and clonus It is expected that the consensus definitions will evolve
Dystonia with time as further information becomes available from
Rigidity basic science and clinical research trials. The existence
Hypotonia
Disorders of inhibition Chorea of the consensus document demonstrates the ability to
Myoclonus reach common definitions between clinicians and
Tics researchers from different fields. We expect that similar
Tremor
Disorders of execution Weakness projects will need to be undertaken in order to define
Bradykinesia hyperkinetic disorders, negative symptoms, and other
Dystonia components of childhood motor impairment. There has
Ataxia
Disorders of planning Apraxia recently been an attempt to re-evaluate the classification
Developmental coordination disorder of cerebral palsy, and this has led to a consensus
Poorly differentiated control statement of algorithms for classification of subtypes of
Disorders of sensation
this particular disorder [38].
532 Movement disorders

Advances in quantifying childhood presumed to reduce spinal motoneuron excitability and

movement disorders thereby reduce the effect of spasticity. Recent studies of
Quantification of movement disorders is important in baclofen given by intrathecal infusion pump have shown
order to be able to determine the effectiveness of substantial success in some cases [51,52]. Dantrolene
therapy in individual children, as well as to determine reduces the effect of muscle contraction. Selective dorsal
the outcomes of clinical research trials. Quantification rhizotomy reduces the effect of spindle afferents on
can be performed at different levels of the NCMRR motoneuron pools and a recent review [53 .] suggested
scale, and measures tend to reflect either the degree of benefits for appropriately selected children. Botulinum
impairment, functional limitation, or disability. toxin reduces the effect of motor neurons on muscle
fibers, and may also reduce the contraction of spindle
Tests of impairment usually are directed at measuring fibers and afferent feedback from stretch receptors. The
hypertonic symptoms [39,40]. Dyskinetic, ataxic, or advent of botulinum toxin therapy for children has led to
other symptoms have been less well studied. Certainly substantial benefits for many children, and this has been
strength is measurable, and kinematics and dynamics verified in several functional outcome studies, reviewed
have been best evaluated in the context of gait analysis by Graham et al. [54].
[41,42]. Rating scales for dystonia have been developed
in adults and children [43 .,44]. Dystonia has proven to be resistant to treatment in many
cases. Trihexyphenidyl has long been the mainstay of
There has been significant recent progress in the treatment in adults and children [55], and blinded
development of functional measures of motor perfor- clinical trials in adults showed that it is probably
mance in children. Recent scales include the Melbourne efficacious at doses much higher than originally recom-
[45,46] and QUEST (Quality of Upper Extremity Skills mended for the treatment of idiopathic Parkinson’s
Test) [47] scales for testing the upper extremity. The disease [56,57]. The use of trihexyphenidyl in children is
GMFM (Gross Motor Function Measure) and GMFCS more recent, with a retrospective study [58] document-
(Gross Motor Function Classification System) are vali- ing its probable effectiveness. The mechanism of action
dated scales that can predict long-term function [48 . .]. is unknown, and it does appear that, as for adults, the
doses in children need to be substantially higher than
There has also been significant progress in the develop- originally recommended. Tetrabenazine has been help-
ment of disability outcome ratings. These ratings ful in the treatment of tardive dystonia, but its
attempt to measure a child’s performance of activities effectiveness in childhood primary or secondary dystonia
of daily living, or participation in age-appropriate has been disappointing [59–64]. The discovery of dopa-
activities. Outcome scales were reviewed by Lollar et responsive dystonia (Segawa’s disease [17]) has led to
al. [23]. many children receiving trials of dopaminergic medica-
tion prior to the initiation of other therapy. One
Advances in therapy consequence of this has been the observation that many
Medical therapy for movement disorders has tradition- children with cerebral palsy or other motor disorders
ally been more successful at reducing positive symptoms have responded to dopaminergic medication despite the
than it has been at improving negative symptoms. This lack of a consistent theory to explain why this might be
does not represent an essential limitation in the ability to so. An open-label trial has shown very encouraging
improve impairment, but rather represents the effects results [65] and a controlled clinical trial is now under
and limitations of medical and surgical intervention. way. One of the most exciting advances in adult
Nevertheless, there have been significant improvements idiopathic torsion dystonia has been the discovery that
in our understanding of treatment of positive symptoms deep-brain stimulation of the internal globus pallidus can
[49]. lead to substantial improvement. This discovery follows
a long tradition of stereotactic neurosurgery for dystonia
Spasticity has long been considered to be a primary [66,67], and lesions in the thalamus may also be effective
determinant of disability. Recently, questions have been [68]. There is increasing evidence that deep-brain
raised as to whether the degree of spasticity is in fact stimulation of the internal pallidum may also be helpful
closely related to the degree of disability [50]. Certainly, in children with both primary (genetic) and secondary
extreme spasticity can lead to an immovable joint and dystonias, although the results in secondary dystonias
therefore reduction could be expected to improve have been less successful [69–71]. This intervention
function. It is more difficult to predict the effects of requires significant further study, and the long-term
intervention in milder cases. Medical therapy of effectiveness of deep-brain stimulation or stereotactic
spasticity has been directed at multiple components of lesions has not yet been adequately investigated.
monosynaptic or polysynaptic spinal reflex pathways. Although originally developed for the treatment of
Medications such as baclofen, clonidine, or tizanidine are spasticity, there is recent evidence that intrathecal
 Pediatric movement disorders Sanger 533

baclofen may also have benefit in some children with Conclusion

dystonia [72–74]. Childhood movement disorders remain a frustrating set
of symptoms. Nevertheless, there has been significant
Treatment of both chorea and myoclonus in children recent progress in classification, measurement, and
has been frustratingly difficult, and there are no treatment of these disorders. There is a tremendous
published controlled trials. Both of these disorders need to continue these efforts in order to provide an
occur frequently in the context of encephalitis or post- accurate and consistent classification for the different
encephalitis, and treatment is usually directed at the components that lead to disability. This effort is not the
underlying disease. Piracetam has long been available province of any one clinical specialty, but needs to be a
in Europe for the treatment of myoclonus, and the joint effort between all the specialties that are concerned
introduction of the related medication levetiracetam in with the treatment of motor-impaired children. There is
the United States suggests that this may provide a new a tremendous need for blinded clinical trials to evaluate
therapy. The role of serotonin precursors or agonists in the effect of the interventions that are available today.
childhood myoclonus is not known, although there Ultimately, we must work to develop and test new
have been reports of success in the treatment of adults. therapies that can eventually lead to meaningful
Chorea in children is most commonly treated with improvement in functional outcome for affected chil-
medications that potentiate or stimulate g-aminobutyric dren.
acid (GABA) receptors, including clonazepam and
valproate. In adults, chorea may be susceptible to Acknowledgements
treatment with dopamine receptor antagonists or tetra- The author wishes to thank Dr Anthony Lang for his comments on the
manuscript. This work was supported by the National Institutes of
benazine, although there have not been studies in Neurological Disorders and Stroke under award K23-NS41243 and by
children [59,60]. Thalamic deep-brain stimulation is a the Stanford University department of Neurology and Neurological
promising modality that will require further studies Sciences.
[75].

Perhaps the most exciting advances in the treatment of References and recommended reading
Papers of particular interest, published within the annual period of review, have
childhood motor disorders have come from non-medical been highlighted as:
.
interventions. There has long been concern that ..
of special interest
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