Case Report

Early-Onset Dystonia after Supplementary Motor
Area Infarction
Kazutaka Nishimura, MD, Toshiyuki Uehara, MD and Kazunori Toyoda, MD

A 63-year-old patient with the right supplementary motor area infarct developed
early-onset dystonia in the left upper extremity. The mechanisms involved in the genesis of focal dystonia are discussed with emphasis on cortical basal ganglia circuit and
efferent projections from the supplementary motor area. Key Words: Movement
disorders—dystonia—infarction—supplementary motor cortex—acute onset.
Ó 2014 by National Stroke Association

Case Report
A 63-year-old man presenting with sudden-onset stiffness of the left limbs was admitted to our hospital. He had
a history of right putaminal hemorrhage 10 years earlier
but no family history of either involuntary movement or
illicit drug use.
On admission, neurologic examination showed disorientation, mild dysarthria, and left central facial palsy. In
addition, posture of the left limbs was characterized by
flexion at the elbow, pronation of the forearm, flexion at
the wrist with grasping, and extension of the foot.
Blood testing including serum copper, ceruloplasmin,
and magnesium were normal. Acute infarctions in the
right supplementary motor area (SMA) and left precentral gyrus were identified on diffusion-weighted imaging
(Fig 1, A). T2*-weighted imaging revealed old hemor-

From the Department of Cerebrovascular Medicine, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan.
Received August 23, 2013; revision received September 25, 2013;
accepted September 27, 2013.
This work was supported by JSPS (Japan Society for the Promotion
of Science) KAKENHI (grant number 24591309).
K.N. contributed in writing the article, and T.U. and K.T. contributed in the critical revision of the manuscript for important intellectual content.
Address correspondence to Toshiyuki Uehara, MD, Department
of Stroke Care Unit, National Cerebral and Cardiovascular Center,
5-7-1 Fujishiro-dai, Suita, Osaka 565-8565, Japan. E-mail: tuehara@
ncvc.go.jp.
1052-3057/$ - see front matter
Ó 2014 by National Stroke Association
http://dx.doi.org/10.1016/j.jstrokecerebrovasdis.2013.09.028

rhage in the right putamen and left thalamus (Fig 1, B).
Occlusion of the left internal cerebral artery at its origin
was observed on magnetic resonance angiography.
Dystonic posture of the left upper extremity appeared
on the second day after admission. On day 6, his dystonic
posture was characterized by abduction of the shoulder,
flexion at the elbow, rotation of the forearm, and extension of the fingers (Fig 1, C). This posture was enhanced
during walking. Focal dystonia gradually improved
spontaneously, with no medication. At 1 month after
onset, only mild dystonic posture with flexion at the
elbow persisted.
In a review of the literature involving 56 cases of poststroke movement disorders, dystonia was the second
most common after chorea.1 In addition, the onset of poststroke dystonia is frequently delayed, with a latent period
ranging from 2 weeks to 4 years.1,2
Dystonia is thought to result from increased thalamocortical excitation as a consequence of decreased inhibitory
output from the globus pallidus internus and subthalamic
nucleus to the thalamus. The fact that poststroke movement disorders are relatively rare, even with marked basal
ganglia damage, is explained by the marked plasticity of
the brain within the basal ganglia anatomical networks,
partly as a result of parallel processing, and compensatory
mechanisms that provide certain resilience and protection
against clinically evident loss of motor control.3,4
Efferent projections from the SMA to the putamen,
caudate nucleus, and subthalamus have been found subcortically.5 Dystonia in the present patient could be
considered likely to be because of the damage of efferent
projections from SMA to subthalamus in addition to old

Journal of Stroke and Cerebrovascular Diseases, Vol. 23, No. 5 (May-June), 2014: pp 1267-1268

1267

1268

K. NISHIMURA ET AL.

Figure 1. (A) Diffusion-weighted imaging showing hyperintense signals in the
right SMA and left precentral gyrus.
(B) T2*-weighted imaging showing old
hemorrhage in the right putamen and left
thalamus. (C) Dystonia in left upper
extremity. (Color version of figure is available online.)

putaminal hemorrhage. This case seems to be useful for
clarifying the pathogenesis of dystonia.

References
1. Alarcon F, Zijlmans JC, Duenas G, et al. Post-stroke movement disorders: report of 56 patients. J Neurol Neurosurgery Psychiatry 2004;75:1568-1574.
2. Chuang C, Fahn S, Frucht SJ. The natural history and treatment of acquired hemidystonia: report of 33 cases and re-

view of the literature. J Neurol Neurosurgery Psychiatry
2002;72:59-67.
3. Mehanna R, Jankovic J. Movement disorders in cerebrovascular disease. Lancet Neurol 2013;12:597-608.
4. Ghika-Schmid F, Ghika J, Regli F, et al. Hyperkinetic
movement disorders during and after acute stroke:
the Lausanne Stroke Registry. J Neurol Sci 1997;146:
109-116.
5. Jurgens U. The efferent and afferent connections of the
supplementary motor area. Brain Res 1984;300:63-81.