doi: 10.2169/internalmedicine.7191-21
Intern Med 60: 3805-3808, 2021
http://internmed.jp

【 CASE REPORT 】

Persistent Hemichorea as a Preceding Symptom of Cerebral
Infarction Due to Middle Cerebral Artery Stenosis
Yuki Ueta 1,2, Haruhisa Kato 2, Makiko Naito 2, Takeshi Taguchi 1,2,
Hiroo Terashi 2 and Hitoshi Aizawa 2

Abstract:
We herein report an 84-year-old woman with right middle cerebral artery (MCA) stenosis who presented
with persistent left hemichorea preceding cerebral infarction. She visited our hospital on day 9 after the
hemichorea onset. Magnetic resonance imaging (MRI) showed no acute cerebral infarction. Magnetic resonance angiography revealed right MCA stenosis. Her hemichorea persisted for 19 days and subsequently disappeared. On day 21, she developed left hemiplegia. Repeat MRI revealed a cerebral infarction in the right
putamen. MCA stenosis can present with persistent hemichorea, even in the absence of cerebral infarction.
Persistent hemichorea with MCA stenosis may presage cerebral infarction.
Key words: chorea, hemichorea, cerebral infarction, middle cerebral artery, basal ganglia, putamen
(Intern Med 60: 3805-3808, 2021)
(DOI: 10.2169/internalmedicine.7191-21)

Introduction
Chorea is a symptom characterized by abrupt involuntary
movements resulting from a continuous flow of random
muscle contractions (1). New-onset chorea can be caused by
infections, autoimmune diseases, drug-induced disorders,
metabolic diseases, neurodegenerative diseases, and
stroke (1). Stroke is the most common cause of sporadic
chorea (2), and hemichorea is the most common involuntary
movement disorder with stroke (3). However, persistent
hemichorea is a rare manifestation of the pre-stroke phase.
We herein report a patient with right middle cerebral artery (MCA) stenosis presenting with hemichorea that persisted for 19 days and preceded cerebral infarction. She developed cerebral infarction of the right putamen with facial
palsy and hemiplegia, and her hemichorea disappeared.

Case Report
An 84-year-old woman, who was undergoing treatment
for diabetes mellitus and hypertension complained of persistent involuntary movements in her left arm and leg. She vis-

ited our hospital on day 9 after the onset of involuntary
movements. She had no history of smoking and drinking or
family history of movement disorders. A neurological examination revealed hemichorea on the left side and no other
signs of neurological impairment. Her hemichorea was persistent and was worsened by mental strain or calculation.
Brain diffusion-weighted imaging-magnetic resonance imaging (DWI-MRI) did not show any abnormalities (Fig. 1A).
Fluid-attenuated inversion recovery (FLAIR) MRI showed
white matter lesions around the anterior and posterior horn
of the lateral ventricles (Fig. 1B). FLAIR and T2-weighted
MRI revealed few small perivascular spaces in the bilateral
putamen. Magnetic resonance angiography (MRA) revealed
stenoses of the right MCA and posterior cerebral artery
(Fig. 1C).
Blood tests revealed normal renal, hepatic, and thyroid
functions. Her serum levels of glucose, iron, copper, and ceruloplasmin were also normal. Her serum levels of lowdensity lipoprotein cholesterol, high-density lipoprotein cholesterol, and triglycerides were 94 mg/dL, 51 mg/dL, and
119 mg/dL, respectively. Immunologic tests were negative
for anti-nuclear and anti-cardiolipin-beta2-glycoprotein I
complex antibodies. Since there was no evidence of acute

1

Department of Neurology, Tokyo Medical University Hachioji Medical Center, Japan and ï¼’ Department of Neurology, Tokyo Medical University,
Japan
Received: January 29, 2021; Accepted: April 18, 2021; Advance Publication by J-STAGE: June 12, 2021
Correspondence to Dr. Yuki Ueta, yukiueta@tokyo-med.ac.jp

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DOI: 10.2169/internalmedicine.7191-21

Figure 1. Magnetic resonance imaging (MRI) results on day 9 and day 21 after the onset of hemichorea. The upper panels show the initial MRI findings on day 9 after the onset of hemichorea, and
the lower panels show the MRI findings on day 21. A: Diffusion-weighted imaging (DWI) shows no
acute lesions. B: Fluid-attenuated inversion recovery MRI reveals white matter lesions around the
anterior and posterior horn of the lateral ventricles. C: Magnetic resonance angiography (MRA) reveals stenosis in the distal portion of the M1 segment of the right middle cerebral artery (MCA) (arrow). D, E: DWI reveals a fresh infarct in the right putamen. F: MRA reveals occlusion in the proximal portion of M1 segment of the right MCA.

cerebral infarction and since she refused to take antidopaminergic agents, we did not prescribe any anti-platelet
or anti-dopaminergic agents.
On day 19 after the onset of hemichorea, she developed
left facial palsy. Hemichorea persisted for 19 days and subsequently disappeared. On day 21, she visited our hospital
with left facial palsy and hemiplegia. Her blood pressure
was 113/69 mmHg, pulse rate 55 beats/minute, and body
temperature 36.7℃. Her muscle strength was grade 2 in the
left arm and grade 3 in the left leg on the Medical Research
Council (MRC) Muscle scale. There were no marked differences in the bilateral tendon reflexes, and her plantar reflexes were absent in both of her feet.
Repeat brain DWI-MRI revealed an acute cerebral infarction in the right putamen (Fig. 1D, E). According to MRA,
the stenosis of the right MCA developed into an occlusion
(Fig. 1F). No stenoses or occlusions were observed on a carotid ultrasound.
She was admitted to our hospital and treated with antiplatelet therapy. On day 29, single photon-emission computed tomography (SPECT) using technetium-99m ethylcys-

teinate dimer revealed hypoperfusion in the right MCA territory, including the basal ganglia (Fig. 2A, B). At discharge
on day 75, her muscle strength in the left limb had improved to grade 4 on the MRC scale, but mild dysarthria
and left facial palsy remained.

Discussion
We encountered a patient with right MCA stenosis presenting with hemichorea that persisted for 19 days and preceded cerebral infarction. She developed a cerebral infarction of the right putamen with facial palsy and hemiplegia,
and her hemichorea disappeared.
Hemichorea is a rare manifestation of cerebral vascular
diseases (3, 4), and post-stroke hemichorea can develop
within five days of the stroke onset but usually develops
within the first day of the onset (4). Some case reports have
described hemichorea-associated MCA stenosis without
cerebral infarction (5, 6). However, persistent hemichorea in
patients with MCA stenosis preceding cerebral infarction has
not been reported.

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DOI: 10.2169/internalmedicine.7191-21

Figure 2. Single-photon emission computed tomography images. A, B: Single-photon emission
computed tomography using technetium-99m ethylcysteinate dimer on day 29 after the onset of hemichorea demonstrates hypoperfusion in the territory of the right middle cerebral artery, predominantly in the right basal ganglia (arrow).

Chorea results from dysfunctional neuronal networks interconnecting the basal ganglia and frontal cortical motor areas (1). In these networks, the striatal direct and indirect
pathways modulate the activity of the globus pallidus internus, which controls motor facilitation and inhibition (1). In
previous reports, it has been suggested that hemichorea accompanied by internal carotid artery or MCA stenosis is
caused by hypoperfusion derangement in the contralateral
basal ganglia (5, 7, 8), frontal cortical and subcortical motor
pathways (9, 10), or both (6). Our patient developed cerebral infarction only in the right basal ganglia within the territory of the right MCA. The SPECT analysis after her
stroke revealed hypoperfusion of the right MCA territory,
and her cerebral hypoperfusion was more severe in the right
basal ganglia than in the right cerebral cortex or subcortical
white matter. In this case, we did not evaluate the cerebral
blood flow during hemichorea before the onset of the cerebral infarction. However, we speculate that the patient’s
hemichorea was caused by mild hypoperfusion in the basal
ganglia, which did not develop into cerebral infarction. The
hypoperfusion probably reduced the activation of the striatal
indirect pathway to a greater extent than that of the striatal
direct pathway, resulting in excessive thalamocortical motor
facilitation.
In the present case, persistent hemichorea with MCA
stenosis, which is similar to the limb shaking observed in
temporary ischemic attacks with carotid artery stenosis (11),
preceded cerebral infarction. Previously reported patients
were treated with anti-platelet therapy or surgical reconstruction, and their hemichorea improved (5, 6). In patients with
hemichorea and MCA stenosis, the prompt administration of
anti-platelet agents should be considered to prevent cerebral
infarction. However, further studies are required to determine whether or not anti-platelet agents prevent cerebral infarction in patients with persistent hemichorea and MCA
stenosis.
In conclusion, MCA stenosis without cerebral infarction

can present with persistent hemichorea. Therefore, MCA
stenosis should be a differential diagnosis of hemichorea,
even in the absence of cerebral infarction. Hemichorea may
presage cerebral infarction with MCA stenosis.
Written informed consent was obtained from the patient for
publication of the case history and the accompanying images.
The authors state that they have no Conflict of Interest (COI).

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Intern Med 60: 3805-3808, 2021

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