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Moyamoya disease initially presenting visual field defect.

M K Chu1, I H Lee, D I Kim

  • 1Department of Neurology, Yonsei University College of Medicine, C.P.O. Box 8044, Seoul 120-752, Korea.

Yonsei Medical Journal
|October 25, 2001
PubMed
Summary
This summary is machine-generated.

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Moyamoya disease typically affects anterior circulation first. This case highlights an unusual presentation with initial visual field defects progressing to blindness, indicating advanced posterior circulation involvement.

Area of Science:

  • Neurology
  • Vascular Neurology
  • Neuroimaging

Background:

  • Moyamoya disease is characterized by progressive stenosis of the distal internal carotid arteries and the formation of compensatory collateral networks.
  • Cerebral infarction in moyamoya disease results from reduced blood flow, manifesting as uneven perfusion in the anterior and middle cerebral artery territories.
  • Typically, posterior circulation involvement occurs later, following anterior circulation symptoms in advanced stages.

Observation:

  • This report details an atypical case of moyamoya disease.
  • The patient initially presented with a transient visual field defect.
  • One month later, profound blindness developed, confirmed by cerebral angiography showing advanced moyamoya disease.

Findings:

  • The case demonstrates an unusual initial presentation of moyamoya disease with visual disturbances.

Related Experiment Videos

  • The rapid progression to blindness suggests significant and potentially rapid posterior circulation compromise.
  • Cerebral angiography confirmed advanced moyamoya disease, correlating with the severe visual deficit.

    • Implications:

    • This case underscores the variability in moyamoya disease presentation, particularly concerning initial symptoms and progression speed.
    • Early recognition of subtle neurological deficits, such as visual field defects, is crucial for timely diagnosis and intervention.
    • The findings emphasize the potential for rapid deterioration and severe visual impairment in moyamoya disease, even when initially presenting atypically.