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Related Experiment Videos

Moyamoya disease presenting with chorea.

K Watanabe1, T Negoro, M Maehara

  • 1Department of Pediatrics, Nagoya University School of Medicine, Japan.

Pediatric Neurology
|January 1, 1990
PubMed
Summary

Moyamoya disease can initially present as choreic movements in children, often triggered by emotional stress. This highlights the importance of considering moyamoya disease in the diagnosis of acquired chorea.

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Area of Science:

  • Pediatric Neurology
  • Neurovascular Disorders

Background:

  • Moyamoya disease is a rare, progressive cerebrovascular disorder.
  • It typically affects children and young adults, leading to stroke or transient ischemic attacks.

Observation:

  • Three pediatric cases of moyamoya disease are presented.
  • The predominant initial symptoms were choreiform (involuntary, irregular) movements.
  • Associated symptoms included unsteady gait and clumsiness.

Findings:

  • Choreic movements were recurrent and exacerbated by emotional stimuli.
  • Movements occurred unilaterally or bilaterally and could alternate sides.
  • These findings suggest a unique presentation of moyamoya disease.

Implications:

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  • Moyamoya disease should be included in the differential diagnosis for pediatric acquired chorea.
  • Early recognition of choreiform movements may lead to earlier diagnosis and intervention.
  • This presentation broadens the understanding of moyamoya disease's clinical spectrum.