Clinical and neuroradiological findings of moyamoya disease in Italy.

A total of 34 Italian patients (15 males and 19 females) suffering from Moyamoya disease (MMD) and selected by a questionnaire survey in 12 neuropediatric and neuroradiologic departments were studied in a multicentric study. The onset of the disease appeared either in childhood (27 patients, aged 0-16 years, mean 5.4 years) or in adulthood (seven patients, aged 25-55 years, mean 35 years). The early clinical symptoms consisted of transient ischemic attacks and/or stroke (20 cases), recurrent migraine-like headaches (seven cases), seizures (six cases) and hemorrhage (one case). A total of four familial cases were found. The final diagnosis was based in all cases on the conventional angiographic findings and more recently also on the magnetic resonance angiography (ten patients). The mean lag time between the first clinical manifestation and the angiographic diagnosis was about 2 years. A medical treatment (vasodilators, antiplatelet agents, calcium channel blockers) was followed by 21 patients, while five cases underwent a surgical revascularization. The follow-up ranges from 1 to 15 years (mean 6 years): A motor (16 cases) and/or mental impairment (14 cases) was detected especially in the childhood onset MMD; only one patient died. In nine cases the long-term outcome persisted without neurological deficit.