Pa. Battistella et C. Carollo, CLINICAL AND NEURORADIOLOGICAL FINDINGS OF MOYAMOYA-DISEASE IN ITALY, Clinical neurology and neurosurgery, 99, 1997, pp. 54-57
A total of 34 Italian patients (15 males and 19 females) suffering fro
m Moyamoya disease (MMD) and selected by a questionnaire survey in 12
neuropediatric and neuroradiologic departments were studied in a multi
centric study. The onset of the disease appeared either in childhood (
27 patients, aged 0-16 years, mean 5.4 years) or in adulthood (seven p
atients, aged 25-55 years, mean 35 years). The early clinical symptoms
consisted of transient ischemic attacks and;or stroke (20 cases), rec
urrent 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 angiograph
ic findings and more recently also on the magnetic resonance angiograp
hy (ten patients). The mean lag time between the first clinical manife
station and the angiographic diagnosis was about 2 years. A medical tr
eatment (vasodilators, antiplatelet agents, calcium channel blockers)
was followed by 21 patients, while five cases underwent a surgical rev
ascularization. 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. (C
) 1997 Elsevier Science B.V.