Copyright (c) 1997 Elsevier Science B.V. All rights reserved.
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. 1997 Elsevier Science B.V.
Mots-clés Pascal : Nishimoto Kudo maladie, Italie, Europe, Age apparition, Symptomatologie, Angiographie RMN, Epidémiologie, Diagnostic, Homme, Encéphale pathologie, Système nerveux pathologie, Système nerveux central pathologie, Appareil circulatoire pathologie, Vaisseau sanguin pathologie, Cérébrovasculaire pathologie, Imagerie RMN, Imagerie médicale, Radiodiagnostic
Mots-clés Pascal anglais : Nishimoto Kudo disease, Italy, Europe, Age of onset, Symptomatology, NMR angiography, Epidemiology, Diagnosis, Human, Cerebral disorder, Nervous system diseases, Central nervous system disease, Cardiovascular disease, Vascular disease, Cerebrovascular disease, Nuclear magnetic resonance imaging, Medical imagery, Radiodiagnosis
Notice produite par :
Inist-CNRS - Institut de l'Information Scientifique et Technique
Cote : 98-0006959
Code Inist : 002B17C. Création : 17/04/1998.