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[Moyamoya disease presenting initially with mental disturbance]

M Hosoya1

  • 1Department of Pediatrics, Saku Central Hospital, Nagano.

No to Hattatsu = Brain and Development
|December 12, 1997
PubMed

Insights

Moyamoya disease can manifest with choreic movements and mental disturbances in children, not just typical ischemic symptoms. Surgical intervention improved motor function but not cognitive deficits in one case.

Area of Science:

  • Neurology
  • Pediatrics
  • Neuroscience

Background:

  • Moyamoya disease typically presents in children with transient ischemic attacks.
  • Mental disturbances are rare initial symptoms of Moyamoya disease.

Observation:

  • A nine-year-old girl with Moyamoya disease presented with choreic involuntary movements and mental disturbance since age four.
  • Imaging revealed multiple ischemic lesions and reduced cerebral perfusion, including in the basal ganglia.

Findings:

  • Superficial temporal artery-middle cerebral artery (STA-MCA) bypass surgery resolved the choreic movements.
  • Post-surgery, cerebral blood flow increased, but intelligence and infarction areas remained unchanged.

Implications:

  • Moyamoya disease should be considered in the differential diagnosis of pediatric mental disturbance.
  • This case highlights the varied neurological presentations of Moyamoya disease beyond typical ischemia.

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