A questionnaire was distributed in early 1996 to 160 leading European
neurological, neuro-pediatric and neurosurgical centers to assess the
present status of Moyamoya disease in Europe. The response rate was 43
%. Information was obtained on a total of 168 patients, of whom 110 ha
d presented before 1992, and 58 from 1993 onward. 82% of the patients
were Caucasian. In all other respects, the clinical findings were simi
lar to those observed in Japan. The present study yields an incidence
of 0.3 patients per center per year, which is approximately one-tenth
of the incidence in Japan. Alongside these results, the history of the
recognition and treatment of this disease in Europe is briefly discus
sed. (C) 1997 Elsevier Science B.V.