JICNAR| Journal of the International
Child Neurology Association
A peer reviewed open access e-journal in Child Neurology
Progressive chorea and dystonia associated with a large
arteriovenous malformation
Jeffrey S Raskin
1, Mered Parnes2, Sandi Lam3
1Section of Pediatric Neurosurgery, Riley Hospital for Children, Department of Neurological Surgery,
Indiana University School of Medicine, Goodman Campbell Brain and Spine, Indianapolis, Indiana,
2Pediatric Movement Disorders Clinic, Section of Pediatric Neurology and Developmental
Neuroscience, Baylor College of Medicine/Texas Children’s Hospital, Houston, Texas,3Department of
Neurosurgery, Division of Pediatric Neurosurgery, Baylor College of Medicine/ Texas Children’s
Corresponding author: Sandi Lam; Email: sklam@texaschildrens.org
Received: 14 June 2018
Accepted: 14 October 2019
A 9-year-old male with intellectual disability and epilepsy presenting with a progressive movement disorder characterized
by chorea and dystonia primarily affecting the left face, arm, and leg and attributed to an arteriovenous malformation
centered within the right thalamus with bilateral extension into the basal ganglia, midbrain and pons is reported.
Kewords: dystonia,basal ganglia,arteriovenous malformation.
© Raskin JS; licensee JICNA
Case Report
A nine-year-old male with intellectual disability and
epilepsy presented with a progressive movement disorder
characterised by chorea and dystonia primarily affecting the
left face, arm, and leg.
Neuroimaging revealed a diffuse Spetzler-Martin Grade
IV arteriovenous malformation centered within the right
Figure 1 A) Axial B) coronal and C) sagittal T1 MRI with gadolin-
thalamus with bilateral extension into the basal ganglia,
ium demonstrating extensive, bilateral arteriovenous malformation
midbrain and pons. An associated network of developmen-
within basal ganglia, thalami and midbrain, and obstructive hydro-
tal venous anomalies was noted to coalesce into ectatic deep
drainage, causing obstructive hydrocephalus (Figure 1).
Surgical management was not indicated given the anatomy
and location of the lesion.
dramatic improvement in his level of awareness, without
The patient presented with a stepwise clinical decline
further improvement in his involuntary movements.
over several months, with worsening chorea and dystonia
resulting in loss of function of the left arm, severe left arm
pain, and loss of the ability to ambulate independently (See
Discussion & Conclusion
supplementary file at http://icnapedia.org/s/148). The in-
voluntary movements and arm pain improved following
The pathogenesis of secondary movement disorders is var-
treatment with clonazepam, baclofen, tetrabenazine, and
ied; etiologies include hypoxia, stroke, and kernicterus [1].
hydrocodone/acetaminophen as well as chemodenervation
Movement disorders resulting from congenital vascular mal-
with intramuscular botulinum toxin. The patient developed
formations are relatively uncommon and can be difficult to
somnolence in the setting of worsening hydrocephalus. Fol-
treat. Apart from producing structural abnormalities, which
lowing the ventriculoperitoneal shunt, he experienced a
can physically disrupt normal basal ganglia-thalamocortical
circuits, vascular malformations can further complicate re-
gional dysfunction by vascular steal phenomena [2]. In this
case, surgical and medical management has been helpful.
Intrathecal baclofen therapy may be an option in the fu-
ture should symptoms become refractory to medical inter-
Appropriate consent was obtained from the family for
video use within this publication.
Competing interests
The authors have declared that they have no competing in-
Author contributions
All the authors contributed to data collection and also crit-
ically reviewed the manuscript. The final version of the
manuscript was approved by all the authors.
This is an Open Access article distributed under the
terms of the Creative Commons Attribution License
(http://creativecommons.org/licenses/by/4.0), which per-
mits unrestricted use, distribution, and reproduction in any
medium, provided the original work is properly credited.
The Creative Commons Public Domain Dedication waiver
applies to the data made available in this article, unless
otherwise stated.
Cite this article as:
Raskin JS, Parnes M, Lam S. Progressive chorea and
dystonia associated with a large arteriovenous malfor-
mation. JICNA [Internet].
2019Oct.4. Available from:
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