Brain Injury

ISSN: 0269-9052 (Print) 1362-301X (Online) Journal homepage: https://www.tandfonline.com/loi/ibij20

Despite dystonia: natural history of delayed-onset
pediatric secondary dystonia
Tal Krasovsky, Orly Bar, Uli Nachshon, Abigail Livny, Galia Tsarfaty, Amichai
Brezner & Jana Landa
To cite this article: Tal Krasovsky, Orly Bar, Uli Nachshon, Abigail Livny, Galia Tsarfaty, Amichai
Brezner & Jana Landa (2019) Despite dystonia: natural history of delayed-onset pediatric
secondary dystonia, Brain Injury, 33:7, 952-958, DOI: 10.1080/02699052.2019.1606452
To link to this article: https://doi.org/10.1080/02699052.2019.1606452

Published online: 05 May 2019.

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BRAIN INJURY
2019, VOL. 33, NO. 7, 952–958
https://doi.org/10.1080/02699052.2019.1606452

CASE REPORT

Despite dystonia: natural history of delayed-onset pediatric secondary dystonia
Tal Krasovskya,b, Orly Bara, Uli Nachshona, Abigail Livnyc,d,e, Galia Tsarfatyc,e, Amichai Breznera, and Jana Landaa,e
a
Pediatric Rehabilitation Department, Sheba Medical Center, Tel Hashomer, Ramat Gan, Israel; bDepartment of Physical Therapy, Faculty of Social
Welfare and Health Sciences, University of Haifa, Haifa, Israel; cDepartment of Diagnostic Imaging, Sheba Medical Center, Tel Hashomer, Ramat Gan,
Israel; dJ. Sagol Neuroscience Center, Sheba Medical Center, Tel Hashomer, Ramat Gan, Israel; eSackler Faculty of Medicine, Tel Aviv University, Tel
Aviv, Israel

ABSTRACT

ARTICLE HISTORY

Background: Dystonia is a movement disorder involving involuntary movements and/or postures of the
limbs, trunk, neck or face. Secondary dystonia following brain injury is uncommon, with unfavorable
long-term consequences. Given the limited evidence regarding pediatric secondary dystonia, this study‘s
aim was to document the natural history of the condition and the effect of interventions on its
symptomatology.
Methods: We describe three cases of girls (age 8 to 11 y) who developed dystonia secondary to an
acquired brain injury, received intensive rehabilitation treatments and were followed for 8–33 months
post-injury. In all three cases, secondary dystonia appeared 1–3 months post-insult.
Results: In none of the cases was there alleviation of dystonic symptoms over time despite physical and
pharmacological interventions; in two cases the dystonic hand is now used as an assisting hand only,
whereas in the third it is completely non-functional. However, despite their impairment, two girls
achieved basic functional independence and one is partly independent in activities of daily living.
Conclusions: Rehabilitation professionals who work with pediatric patients susceptible to developing
secondary dystonia should be aware of its possible consequences and inform families and staff.
Intensive task-specific training during rehabilitation may be key to regaining overall functional capabilities despite residual impairment.

Received 26 July 2018
Accepted 8 April 2019

Introduction
Dystonia is a movement disorder involving involuntary movements and/or postures of the limbs, trunk, neck or face (1).
Dystonia may arise from genetic disorders (“primary”), or, less
frequently (2) from peri-natal ischemia, infections, head trauma
(3), medications or metabolic diseases (“secondary”) (4).
Secondary dystonia usually is associated with lesions of the basal
ganglia or the thalamus (5) often following ischemic stroke (6). In
children, this phenomenon may manifest months or years after the
original brain insult, and the mechanism underlying its appearance is still unclear (7). Treatment options include pharmacotherapy (8), botulinum neurotoxin injections (9), deep brain
stimulation (10) and physical and occupational therapy (4).
Management of pediatric secondary dystonia is especially challenging since both pharmacological treatments and deep brain stimulation are less effective in children with secondary, compared
with primary dystonia (11). Given the detrimental long-term
consequences of dystonia on activities of daily living and quality
of life (12), documenting the natural history of pediatric secondary
dystonia and the effect of interventions on its symptomatology is
essential. Thus, the current work presents the long-term outcomes
of three children who developed delayed secondary dystonia subsequent to brain injury. The three girls, with diverse patterns of
injury etiology and symptom progression, underwent rehabilitation in a Pediatric Rehabilitation department and were evaluated

KEYWORDS

Rehabilitation; acquired
brain injury; hand function;
movement disorders; case
report

longitudinally using outcome measures addressing the different
levels of the International Classification of Functioning, Disability
and Health (ICF (13)). In absence of a clinical guideline for
treatment of pediatric secondary dystonia (14), the aim of the
current work is to evaluate their long-term progression in the
different domains in light of the treatment received.
The three girls were admitted to Pediatric Rehabilitation
approximately 1-month post-insult. During inpatient hospitalization, they received intensive physical and occupational therapy
(daily sessions 5 times a week) as well as speech therapy, psychotherapy, and familial support. During outpatient hospitalization, treatment intensity was gradually decreased from 3 to 2
times a week. During therapy sessions, all three were treated with
a therapeutic approach composed of the same principles: passive
movement, strength training, somatosensory training (including
use of neoprene splints) and task-specific training focused on
function (15). Several strategies were attempted for task-specific
functional training; (1) a computerized biofeedback system for
upper limb movement (16), (2) a computerized kinect-based
virtual reality system (17), (3) repetitive motor training intended
to exit dystonic patterns, and (4) contextual functional training
using everyday objects (e.g. a key, a bottle, a toothbrush, etc.).
Bilateral arm use was encouraged during training when possible.
The girls were followed for 8–33 months post-injury and
underwent routine evaluations of function according to the different levels of the ICF (Table 1): muscle strength (grip, pinch;

CONTACT Tal Krasovsky
tkrasovsk@univ.haifa.ac.il
Department of Physical Therapy, Faculty of Social Welfare and Health sciences, University of Haifa, Israel
Color versions of one or more of the figures in the article can be found online at www.tandfonline.com/ibij.
© 2019 Taylor & Francis Group, LLC

BRAIN INJURY

body function and structures domain), gross motor skill (Box and
Blocks test (18)) and fine motor skill (Nine Hole Peg Test (19)),
both in the activity domain of the ICF (capacity). In addition,
activity performance and participation capabilities were assessed
using the Hebrew version of the Pediatric Evaluation of Disability
Inventory (PEDI) (20,21). The PEDI is a widely used criterionand norm-referenced test of performance capabilities which is
further divided into three subscales: functional skills (what the
child can do), caregiver assistance (how much help the child is
given) and social function (which was not addressed here since the
children were in the hospital during test administration). For each
subscale, performance capability is assessed for different activities.
For the current work, PEDI functional skills and caregiver assistance scores for mobility (59 items) and self-care (73 items) were
transformed into age-independent normative scaled scores, ranging from 0 to 100. The mean PEDI score within each age group is
50, and ±2SD from the mean (scores of 30–70) include 95% of
children. A change of 11 points on the PEDI scaled score is
considered clinically meaningful (22). Written informed consent
was obtained from families according to the hospital‘s ethics
review board.
Case no. 1
BB was an 11 y old girl when she was involved in a car accident as
a pedestrian. She was diagnosed with diffuse axonal injury (spanning both hemispheres and involving right frontal areas, right
midbrain, and right corticospinal tract) and hemorrhage in the

953

frontal lobe, left corpus callosum, left posterior horn of lateral
ventricles and left thalamus (Figure 1(a)). Upon admission to
rehabilitation, she had left hemiparesis (with no sensory deficits)
but was able to grasp objects with her right (dominant) hand. She
also had oral pharyngeal apraxia and mutism. She was completely
dependent in all self-care activities (PEDI functional skills self-care
: 41.2, mobility: 29.0; caregiver assistance self-care: 35.0, mobility:
40.9). In rehabilitation, her left (hemiparetic) arm improved in
strength and control. BB was discharged from inpatient hospitalization 5 months post-injury, with PEDI functional skills scores of
65.3 (self-care) and 71.6 (mobility) and caregiver assistance scores
of 66.9 (self-care) and 66.7 (mobility), indicating partial independence in activities of daily living (ADL).
Dystonia in her right hand was noticed 2 months postinjury as a static flexor posture in her right fingers. While
proximal muscle strength in her right arm gradually
improved, it was extremely difficult for her to extend her
fingers. BB refrained from using her right hand except as an
assisting hand, and hand dominance was fully transferred to
the left within several weeks. Simultaneously, a dystonic posture appeared in her jaw and she was able to close her mouth
only with assistance. Her oral dyspraxia worsened, and drooling appeared with effort. Seven months from injury, when
discharged from inpatient hospitalization, BB was partly independent in ADLs (PEDI functional skills scores of 66.8 (selfcare) and 79.8 (mobility) and caregiver assistance scores of
69.6 (self-care) and 68.5 (mobility)). However, the dystonic
postures persevered, with involvement of the elbow and

Table 1. Evaluation of function in three representative time points for the three cases. The numbers represent raw score for each evaluation. In brackets: the number
of standard deviations from age- and gender referenced norms (z-score).
Time from admission (days) Dominant side (dystonic) – Score (z-score) Non-dominant side (not dystonic) – Score (z-score)
17
13.33 (−2.46)
20.00 (−1.93)
220
20.00 (−1.76)
30.00 (−0.8)
449
9.0 (−4.19)
28.3 (−2.01)
Lateral pinch (lb)
17
2.33 (−3.7)
4.60 (−2.17)
220
5.00 (−2.43)
10.00 (0.08)
449
1.0 (−4.3)
7.6 (−0.9)
Box and Blocks (N)
17
0
34 (−3.91)
220
0
46 (−2.51)
449
4 (−7.4)
51 (−1.93)
Nine Hole Peg Test (sec)
17
Unable
48 (9.4)
220
Unable
30 (3.5)
449
Unable
29 (3.2)
No. 2 Grip strength (lb)
0
18.3 (−0.67)
23.3 (−0.06)
65
20.0 (−0.46)
32.3 (1.08)
237
20.0 (−0.46)
30.0 (0.78)
Lateral pinch (lb)
0
7.3 (−0.36)
6.0 (−0.68)
65
6.3 (−0.76)
8.0 (0.04)
237
8.0 (−0.08)
9.0 (0.39)
Box and Blocks (N)
0
38 (−4.86)
49 (−2.19)
65
34 (−5.65)
49 (−2.19)
237
24 (−7.61)
45 (−2.96)
Nine Hole Peg Test (sec)
0
36 (9.1)
27 (1.8)
65
35 (8.6)
26 (1.5)
237
60 (21.7)
29 (2.4)
No. 3 Grip strength (lb)
28
32.7 (0.77)
41.7 (2.06)
98
31.7 (0.6)
44.0 (2.38)
1016
16.7 (−1.8)
46.7 (2.74)
Lateral pinch (lb)*
28
9.7 (0.48)
10.8 (0.73)
Box and Blocks (N)
28
40 (−4.47)
48 (−2.38)
98
12 (−9.96)
49 (−2.19)
1016
18 (−8.78)
58 (−0.46)
Nine Hole Peg Test (sec)
28
39 (10.7)
27 (1.8)
98
85 (34.9)
20 (−0.4)
1016
80 (32.2)
25 (1.0)
Case
Evaluation
No. 1 Grip strength (lb)

*Two measurements of lateral pinch are missing for technical reasons.

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T. KRASOVSKY ET AL.

Figure 1. (a) MRI scan of BB taken 3 months following injury. Axial slices, Top row: a T2 gradient echo (GRE) scan, sensitive to paramagnetic blood products
demonstrating small regions of hypointense lesions in the left and right frontal lobes, left thalamus and basal cistern. Bottom row: Fluid-attenuated inversion
recovery (FLAIR) hyperintense foci in the same regions. (b) Evaluations of hand function for BB. 9HPT = Nine hole peg test. A red arrow marks occurrence of
a Botulinum Toxin injection and its effect on grip force.

shoulder. During the effort, the arm assumed a dystonic posture with 90 degrees abduction of the shoulder, shoulder
hyperextension and full flexion in the elbow, wrist, and
fingers.
During rehabilitation, BB underwent botulinum toxin
injections (Dysport; arm and hand: 275 units, jaw: 40
units) every three months to right arm muscles (deltoid,
biceps brachii, pronator teres), hand muscles (opponens
policis) and jaw muscles (digastric, pterygoid). Finger flexor
muscles and lumbricals were injected and resulted in
further reduced grip function (see, for example, Figure 1
(b), red arrow: a reduction of ~50% in grip force following
a Dysport injection, from 18.33lb to 10lb; a concurrent
decrease in pinch strength was noted, from 5.16lb to
2lb – not in figure). However, BB‘s right grip force during
rehabilitation achieved 20lb at most, which is still 1.76
standard deviations below age norms and lateral pinch
was at most 5.6lb, 2.35 standard deviations below age
norms. Thus, these limited forces were not considered
functional and the injections to finger muscles as well as
to distal arm muscles were continued considering their
cosmetic value in alleviating the dystonic posture.
Proximal injections to the arm were ineffective and were
stopped. In contrast, injections to the jaw were continued
and considered effective in enabling the mouth to close. BB
received Tetrabenzine (25 mg, twice daily), which was
replaced by Trihexyphenidyl (1 mg, twice daily) after 3
months due to lack of effect and a side effect of sleepiness.
After 1 year, Trihexyphenidyl was discontinued as well due
to lack of change in function. During sessions, bilateral
functional training was emphasized and the use of the
dystonic hand as an assisting hand was encouraged. The
results of her functional arm evaluations are depicted in

Figure 1(b). BB attended outpatient rehabilitation for 16
months. Two years post-insult, she was able to use her
dystonic hand as an assisting hand with a partial power
grip (both power and pinch grip were over two standard
deviations below age norms). Despite some return of grip
force in her right hand (Figure 1(b)), the right arm did not
regain either gross or fine motor function.
Four years post-insult, at age 15, BB is still limited in
communication and eating due to her motor speech disorder
(hyperkinetic dysarthria) associated with her dystonia. She is
still mute. She still receives hydrotherapy, physical and language therapy (2–3 times a week), but further deterioration in
her use of the dystonic hand occurred and her dystonic arm is
completely non-functional, not even as an assisting hand.
However, according to parental report, she is fully independent in ADLs and attends a regular school with some
modifications.
Case no. 2
EL was an 8 y old girl when she was hospitalized with acute
ischemic left MCA stroke. An MRI revealed ischemic damage
to the left frontal lobe, left basal ganglia, mainly putamen and
globus pallidus (Figure 2(a)). EL Initially had right hemiparesis and central facial palsy, with slightly increased muscle
tone in her right arm (with no sensory deficits). During her
hospitalization (2 months as an inpatient, 1 year as an outpatient) the increased tone disappeared and muscle strength
in her right arm returned to normal. She was independent in
basic ADLs, with a PEDI functional skills self-care score
which increased from 74.7 on admission to 93 after 2 months.
Her PEDI mobility score was 100 since admission. Caregiver
assistance scores increased from 76.7 (self-care) and 85

BRAIN INJURY

955

Figure 2. (a) MRI scan of EL taken following injury. Axial slices, Right: Two right images, taken immediately after injury, demonstrate restriction in diffusion weighted
images in the territory of the left MCA including the left basal ganglia, in acute stage. Left: T2-FLAIR image, taken 6 months post-injury, demonstrates the chronic
damage in the same regions. (b) Evaluations of hand function for EL. 9HPT = Nine hole peg test.

(mobility) on admission to 83.2 (self-care) and 100 (mobility)
after 2 months, indicating a high level of functional independence in self-care activities and complete independence in
mobility.
A dystonic posture in her right (dominant) arm appeared
upon release from hospital (2 months post-insult), characterized by full elbow flexion with wrist extension and thumb
flexion. She was initially able to exit this posture and performed bilateral movements, albeit slower and less precise, as
can be seen in functional evaluations such as the Box and
Blocks or 9 Hole Peg Test (Table 1, Figure 2(b)). Her grip and
pinch force were within norms. In addition to the arm, EL
developed dystonia in her right foot (dorsiflexion of the big
toe) which resulted in a change in her gait pattern (right foot
inversion, dragging of the foot). Five months post-insult, the
dystonic arm pattern was extended to shoulder abduction and
extension and hand dominance was fully transferred to the
non-dystonic hand. It became increasingly difficult for EL to
exit the dystonic posture and use her right arm as an assisting
hand. Use of everyday objects during rehabilitation sessions
and everyday life facilitated the process of exiting the dystonic
posture, but the dystonic patterns worsened when the task
was associated with increased fine motor requirements.
During hospitalization, EL was treated with Aspirin.
Treatment with Tetrabenzine (12.5 mg, twice daily) was discontinued due to side effects (fatigue and mood disorders –
crying). She was since then treated with Trihexyphenidyl
(2.5 mg, twice daily) with a small improvement in function,
but discontinued after 2 months. Botulinum toxin injections
(Dysport; 125 units) were administered to the leg (tibialis
posterior and extensor hallucis longus muscle), relieved toe
dorsiflexion and improved the gait pattern. After 2 years,
treatment effectiveness decreased, and dosage was increased
to 300 units. Injections to the arm were not considered due to
the extent of muscles involvement.

Following discharge from outpatient rehabilitation, EL
continued to receive physical and occupational therapy 1–2
times a week (intermittently). Five years post-insult, EL’s right
hand is used as an assisting hand, and the dystonic pattern is
variable and worsens with effort, both physical (e.g. gait) and
cognitive (attentional demand). When the hand is not in use,
increased tone appears in variable postures. EL’s gait pattern
is still affected by her dystonic foot. However, EL is independent in basic ADLs and attends a regular school (with an
assistant) where she types with her left (non-dystonic) hand
and uses the right hand effectively for stabilization.

Case no. 3
MR was a healthy 9 y old girl when she was diagnosed with
left MCA ischemic stroke several hours following a fall. An
MRI revealed ischemic damage to the left insula, basal
ganglia and corona radiata (Figure 3(a)). She was suspected
with left internal carotid artery dissection which could have
caused the stroke and during hospitalization she was also
diagnosed with hyper-coagulation and is treated with
Coumadin ever since. MR initially presented with right
hemiparesis, central facial palsy, and aphasia. Movement
in her right arm gradually returned. During her rehabilitation (2.5 months as an inpatient and 11 months outpatient),
MR presented with a small change in self-care function and
a larger change in mobility: her PEDI functional skills
scores increased from 66.8 (self-care) and 49.7 (mobility)
to 74.7 and 89.2 three months post-injury. Caregiver assistance scores changed from 63.4 (self-care) and 66.7 (mobility) to 74.5 and 66.7 (i.e. no change in mobility or self-care
level of independence).
A dystonic posture in her right (dominant arm), involving
abduction and internal rotation of the shoulder, flexion of the

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T. KRASOVSKY ET AL.

Figure 3. (a) MRI scan of MR taken immediately following injury. Axial slices, top row: left, FLAIR image; right, T2 image, demonstrating ischemic changes in the basal
ganglia. Bottom: Diffusion weighted imaging demonstrating acute restriction in the basal ganglia. (b) Evaluations of hand function for BB. 9HPT = Nine hole peg test.

elbow and flexion of wrist and fingers, appeared 1.5 months
post-insult during motor effort. In addition, a dystonic pattern of external rotation appeared in her right foot. Associated
movements appeared in the jaw, especially during performance of fine motor skills using her right arm, but these
have decreased with time. Figure 3(b) highlights
a deterioration in gross and fine motor skills during the first
3 months and stabilization since. Grip force in her right arm
decreased somewhat with time. Two years post-insult MR
reported decreased sensation (light touch) in her right arm
(proximally and distally) and in her right foot.
Pharmaceutical interventions were not offered to the
family due to the relative focal nature of the dystonic symptom. Injections of botulinum toxin were offered but rejected
by the family.
Following discharge from outpatient rehabilitation, MR
continued to receive hydrotherapy, physical therapy, and
speech therapy once a week. Three years post-insult, MR is
integrated in a regular school (with an assistant). She is partly
independent in ADLs, and receives help in activities such as
dressing, washing and tying shoelaces. Her right arm is used
only as an assisting hand and the dystonic pattern is prominent. When asked to perform tasks with her right-hand MR is
able to perform both power and pinch grips with limited force
regulation. Flexor tone in the fingers is variable. MR is partly
able to exit the dystonic pattern by using functional cues such
as everyday objects, or cognitive strategies such as “to walk
like a soldier”.

Discussion
Secondary dystonia is extremely rare in adults (23). Although
more common in children, rising up to 21% among children
with basal ganglia stroke (6), literature dealing with secondary
dystonia in children is scarce. Indeed, the experience of
a child manifesting dystonic symptoms >1 month post-insult

can be disheartening for clinicians and families alike. The
current work presents three cases with similar negative prognostic factors: the literature suggests that children with left
side basal ganglia lesions (as two of the cases here) are more
likely to develop secondary dystonia, possibly due to the
lateralization of functions of praxis to the left hemisphere
(24). Girls are more susceptible compared to boys (6) and
younger age at insult may also be associated with increased
symptom severity due to changes in brain metabolism (25).
Thus, in these three cases, the prognosis of dystonia is quite
guarded. Long-term follow-up of these three girls revealed
several common features, despite different injury mechanisms: in all cases, symptom severity increased over time and
response to treatment was generally poor. In the case of BB,
dystonic symptoms were especially prominent and the ability
to exit the dystonic posture was reduced compared with the
other girls. This was probably due to her different injury
mechanism; a complex bilateral diffuse axonal injury with
left thalamic damage due to traumatic brain injury (unlike
basal ganglia involvement with a unilateral lesion following
a stroke for EL and MR). Her diffuse pattern of injury may
involve different cerebello-thalamo-cortical pathways and
may underlie the increased severity of dystonia in this case
and the complete absence of hand use in the long run (26).
Despite the different injury mechanisms, the impact of
dystonia in these three cases was consistent. Furthermore,
our results demonstrated that impact varied according to
the domain of the ICF evaluated. Grip and pinch forces,
measures of impairment, were within age norms in two
cases and unchanging in the third case – in all cases deficits
in strength were unrelated to progression of dystonia. In
contrast, the impact of dystonia on measures of activity
capacity (Box and Blocks, 9HPT) in these girls was profound and increased with time. Finally, in two of the three
cases complete independence in basic ADLs was achieved
over time, and partial independence in the third case – as

BRAIN INJURY

evidenced by their PEDI scores and families‘ report. The
ability to achieve functional independence in the presence
of an aggravating activity limitation can be resolved by
considering two main factors. The first is that in the three
cases presented here, family played a crucial role and was
fully committed to the rehabilitation process (27). Families
assisted, among other things, with environmental modifications required to promote independence (e.g. specialized
devices for one-hand operation of everyday objects).
The second is that in all three cases, functional, meaningful
bilateral task-specific training was used. We propose that
this strategy was effective since it assisted the girls to exit
the dystonic pattern initially, and later, when exiting the
dystonic pattern was more difficult, enabled the use of
compensatory motor patterns which promoted functional
independence even without a significant decrease in dystonic symptoms. Task- and context-specific training are
well-known principles of stroke rehabilitation (15,28),
based on a large amount of evidence supporting the superiority of practice of motor tasks which are relevant to the
patient (29) and providing rehabilitation in the patient‘s
home and natural environment (30). In the prolonged
follow-up following rehabilitation, support was provided
for the girls and their families in setting motor goals relevant to them, specifically in ADLs and instrumented ADLs.
We believe that similar to its role in focal dystonia (31),
task-specific training enabled the transition of the girls to
functional independence in the long run. To a certain
extent, use of everyday objects in rehabilitation facilitated
bilateral arm function and use of mental imagery (e.g.
“walk like a soldier”) improved the gait pattern. Another
important factor to be considered in order to promote
function in the long term is an early transfer of hand
dominance. In delayed-onset movement disorders, hand
dominance transfer is more likely to succeed at a younger
age (25). We suggest that children with secondary dystonia
may benefit from early training for transfer of hand
dominance.
Management of dystonia in rehabilitation is multi-faceted,
geared not only towards improvement of function of the
affected body part, but towards functional improvement of
the body as a whole, as it relates to activities of daily living
and participation in the community. Thus, in the long-term
follow-up of the cases described here, although function in the
dystonic body part(s) gradually deteriorated, the children’s
functional capabilities improved with rehabilitation. This
approach addresses one of the major concerns of families and
children with dystonia (32), i.e. difficulties with ADLs and selfcare. This work also highlights the importance of measurement
of change following rehabilitation at the different levels of the
ICF framework – since measurement solely on impairmentbased scales may limit the appreciation of change over time in
pediatric dystonia (32). An important limitation of the current
work is that community participation and health-related quality of life measures were absent from the case descriptions.
However, over 80% of variance in social participation of children after acquired brain injuries are explained by discharge
self-care scores (33) indicating that some indication of social
status can be inferred from existing scores.

957

Delayed-onset secondary dystonia in children typically
responds poorly to pharmaceutical treatment (34). In the
cases presented here, neither Tetrabenzine nor Trihexyp
henidyl were associated with functional improvements,
and the only pharmacological intervention which generated
functional improvement was botulinum toxin injected to
the jaw in one case and to the leg in another. Botulinum
toxin-A was previously shown to promote hand function in
children with dystonia following acquired brain injury (35),
but the long-term follow-up of the cases presented here
reveals the weakness of botulinum toxin-A, namely
decreasing efficacy and a requirement to increase dosage
to maintain efficacy. Botulinum toxin injections may also
not be effective when the number of muscles to be injected
is high, as was the case for the arm of BB. These factors, as
well as possible weakness due to injections, should be
considered carefully for this course of treatment.
In the three cases described here, several other treatments
were considered but eventually not attempted. Constraintinduced movement therapy and inhibitory casting were not
attempted due to unsuccessful prior experience and lack of
supporting evidence. Deep brain stimulation of the globus
pallidus, which is effective for children with primary dystonia
(36) and cerebral palsy (37) is less effective in secondary
dystonia in adults (38) and lacks supporting evidence in
children (39), and thus was not used in these cases.
In conclusion, the three cases described here demonstrate
a guarded prognosis for secondary dystonia in children, with
limited effectiveness of interventions targeted at motor
impairment of the dystonic limb. Rehabilitation experts who
face the onset of secondary dystonia in children need to
acknowledge its implications and prepare the child, family,
and environment for possible functional outcomes of secondary dystonia. However, in order to achieve functional independence in these cases, we propose that clinicians focus on
context- and task-specific training along with early transfer of
hand dominance. Based on our experience, this rehabilitation
approach can promote function in the long term in these rare
cases.

Disclosure Statement
The authors declare they have no conflict of interest.

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