The manifestations of moyamoya disease in children and adults and the results of various surgical procedures are reviewed from the Department of Neurological Surgery and Section of Child and Adolescent Neurology, Mayo Clinic, Rochester, MN. Onset is in the first or the fourth decades of life. Of 518 patients registered in Japan, 155 were children <15 years, and 234 were adolescents or adults. In children, recurrent ischemic attacks (in 39%), strokes (in 39%), and seizures (14%) were the most common features, whereas in adults, hemorrhage (65%), and strokes (18%) were most frequent. Mortality was 7.5% for the total series; 10% for adults and 4.3% for children. Intracranial bleeding was the cause of death in 5 of 9 children (55%) and in 19 of 30 adults (63%). Of 27 children with only TIAs who were untreated, the TIAs gradually resolved but more than one-half showed cognitive impairment at 5 to 10 year follow-up. Ischemic symptoms diminished in 10 Mayo patients treated surgically at 13.5 years (mean age), using STA-MCA anastomosis (5), EDAS (2), and EDAS/EMS (3). Most published reports find surgical intervention of benefit in children but not in adults. [1]

COMMENT. The natural history of moyamoya disease is characterized by neurologic deterioration, strokes and hemorrhage, seizures, and mental deterioration. Current evidence favors surgery to revascularize ischemic brain tissue by collateral pathways, especially in children with ischemic symptoms.

An 11 year-old girl with acute unilateral chorea as the presenting manifestation of moyamoya disease is reported from Hospital Plaza de Cruces, Vizcaya, Bilbao, Spain [2]. Chorea resolved after 15 days haloperidol therapy, and no further ischemic episodes had occurred in 16 months during treatment with nicardipine. The authors cite two previous reports of moyamoya and chorea.