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A case of isolated middle cerebral artery stenosis with hemichorea and moyamoya pattern collateralization

Seok Jong Chung1, Hyung Seok Lee1, Han Soo Yoo1

  • 1Department of Neurology, Yonsei University College of Medicine, Seoul, Korea.

Journal of Movement Disorders
|May 29, 2014
PubMed

Insights

This case study presents an atypical presentation of moyamoya disease in a young woman with isolated middle cerebral artery stenosis. Bypass surgery effectively resolved her hemichorea by restoring vascular flow.

Area of Science:

  • Neurology
  • Vascular Neurology
  • Neuroimaging

Background:

  • Isolated middle cerebral artery (MCA) stenosis in young adults without comorbidities may represent a distinct pathological entity.
  • Moyamoya disease typically involves progressive stenosis from the internal cerebral artery (ICA) to other intracranial vessels.

Purpose of the Study:

  • To report an unusual case of moyamoya disease presenting as isolated MCA stenosis with hemichorea in a young woman.
  • To evaluate the effectiveness of bypass surgery in managing vascular insufficiency and hemichorea in this atypical presentation.

Main Methods:

  • Clinical presentation of choreic movements.
  • Brain magnetic resonance imaging (MRI) to rule out stroke.
  • Conventional angiography to assess MCA stenosis.
  • Single photon emission computed tomography (SPECT) with acetazolamide for perfusion assessment.
  • Follow-up angiography.
  • Surgical intervention (bypass surgery).

Main Results:

  • A 26-year-old woman presented with hemichorea and 48% left M1 stenosis without ICA stenosis.
  • SPECT revealed perfusion asymmetry.
  • One-year follow-up angiography showed complete left M1 occlusion with neovascularization, suggestive of moyamoya disease.
  • Bypass surgery led to complete resolution of hemichorea.

Conclusions:

  • This case suggests moyamoya disease can present atypically with isolated MCA stenosis and hemichorea.
  • Bypass surgery is an effective treatment for vascular insufficiency and hemichorea in such cases.
Keywords:
HemichoreaMiddle cerebral artery stenosisMoyamoya

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