Clinical/Scientific
Notes

Tao Xie, MD, PhD
Issam Awad, MD
Un Jung Kang, MD
Peter Warnke, MD

DBS REDUCED HEMICHOREA ASSOCIATED
WITH A DEVELOPMENTAL VENOUS ANOMALY
AND MICROBLEEDING IN STN

Developmental venous anomaly (DVA) is the most
common vascular malformation and is usually asymptomatic. Movement disorders associated with DVA
have rarely been reported, except a case of hemichorea
and hemiballism caused by DVA in the contralateral
putamen associated with hyperglycemia.1 The abnormal movements in that case nearly resolved after treatment with insulin and risperidone. We report a case of
DVA who presented with hemichorea without metabolic derangement but microbleeding surrounding the
DVA in the contralateral subthalamic nucleus (STN)
area. The lesion was unresectable and deep brain stimulation (DBS) of globus pallidus interna (GPi) helped
control the pharmacologically refractory symptoms.
This study provides Class IV evidence. It is a single
observational study without controls.
Case report. A 22-year-old Caucasian man presented
to our clinic with a 4-year history of hemichorea, with
some myoclonic dystonic features, which started in his
left hand and then gradually involved his whole left
arm and left leg as well as his face to a lesser extent.
His left hand function was severely impaired and his
gait was mildly unsteady with occasional falls. He
also felt socially embarrassed. He had no family
history of neurologic diseases. He had type I diabetes,
which was well-controlled by an insulin pump.
On examination, he had no other motor or sensory deficit. A brain MRI/magnetic resonance angiography showed a large DVA in the right cerebral
peduncle–substantia nigra–STN–thalamic areas,
which was confirmed by cerebral angiography.
MRI VenBold also demonstrated punctate foci of
susceptibility in the right STN and substantia nigra
areas, suggesting microbleeding and small cavernous
malformations (CMs) (figure, A and B). He was
then treated with the maximum tolerated dose of
clonazepam 2 mg TID, trihexyphenidyl 4 mg
TID, and tetrabenazine 37.5 mg TID, but without
benefit. He declined trying neuroleptics. Surgical
resection was not an option given its location.
After a thorough discussion among the patient,
our DBS team, and the neurovascular team, DBS

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was pursued. A single trajectory DBS electrode
was successfully placed to the right posteroventral
GPi (stereotactic parameters related to the anterior
and posterior commissure line were x/y/z of 121.9/
12.2/22.1 mm) guided by the fusion of stereotactic
head CT with MRI and a superimposed digital Schaltenbrand Atlas. The trajectory and functional target
were carefully selected so as to avoid the cerebrovascular anomaly, with the final target 7 mm lateral to the
most lateral part of the DVA (figure, C). There was
good suppression of the abnormal movements in
response to intraoperative macrostimulation. Postoperatively, his symptoms were well-suppressed by the
DBS contacts of C1/12 over the course of 10 months
follow-up so far (figure, D and E, illustrating his
improvement in drawing an Archimedes spiral after
DBS at his 10-month postoperative visit, on 3.6
volts/120 ms/60 Hz). He responded better at 60 Hz
than 130 Hz.
Discussion. DVA can have concomitant CM or telangiectasia,2,3 thrombosis with venous ischemia, or hemorrhage.4 There was only one previously reported case
of abnormal movement caused by a DVA around the
putamen area associated with hyperglycemia.1 CMs of
basal ganglia were previously reported in association
with movement disorders.5 We report the first case of
a movement disorder associated with DVA in the STN
area, which was likely triggered by microbleeding associated with concomitant CM. Since our case involved a
predominant DVA in an eloquent area and microbleeding rather than an overt CM, a resection strategy
was not possible. Our case responded well to GPi DBS
treatment. The improvement in motor performance
was possibly associated with an increase in activation
of those cortical areas involved in motor decisionmaking (supplementary motor area and anterior
cingulate cortex) and execution (sensorimotor area),
as demonstrated in a previous report using bilateral
GPi DBS to improve chorea and motor performance
in Huntington disease.6
On one hand, our case should raise the awareness of
DBS treatment option in movement disorders caused
by DVA and vascular complications in the basal ganglia, as the pharmacologic treatment of the movement
disorders caused by these relatively common structural
lesions could be difficult. On the other hand, we also

Figure

Brain imaging and spirals before and after deep brain stimulation

Brain MRI VenBOLD (A) and Sag Reformat (B) imaging before deep brain stimulation (DBS) placement show developmental
venous anomaly and microbleeding, and head CT after DBS placement shows globus pallidus interna DBS location (C). Spirals drawn before (D) and 10 months after (E) DBS treatment show significant improvement in hand function with DBS
treatment.

have to bear in mind that the DBS electrode trajectory
and target should be chosen carefully so as to avoid the
vascular anomaly, which was possible in this case, but
may not always be possible with DVA or other cerebrovascular anomalies with complications causing
movement disorders.
From the Department of Neurology (T.X.) and Department of
Neurosurgery (I.A., P.W.), University of Chicago Medical Center,
Chicago, IL; and Department of Neurology (U.J.K.), Columbia
University Medical Center, New York, NY.
Author contributions: Tao Xie: drafting and revising the manuscript
for content and analysis of data, intraoperative monitoring, DBS
programming. Issam Awad: revising the manuscript for content
and analysis of data. Un Jung Kang: revising the manuscript for
content and analysis of data. Peter Warnke: revising the manuscript
for content and analysis of data, performing the surgery.
Study funding: No targeted funding reported.
Disclosure: T. Xie and I. Awad report no disclosures. U. Kang is on
the medical advisory board of CVS/Caremark. P. Warnke reports
being Associate Editor of the Journal of Neurology, Neurosurgery
and Psychiatry. Go to Neurology.org for full disclosures.
Received June 26, 2013. Accepted in final form October 14, 2013.

Correspondence to Dr. Xie: txie@bsd.uchicago.edu
© 2014 American Academy of Neurology
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