MOYAMOYA DISEASE IN PATIENTS OF FINNO-UGRIC ORIGIN

The terms moyamoya disease, moyamoya syndrome and moyamoya phenomenon can be found dispersed throughout the literature. The diagnostic crite ria for moyamoya disease are: (1) stenosis or occlusion of the anterio r cerebral, middle cerebral and internal carotid arteries, (2) an abno rmal vascular network near these arteries and (3) bilateral findings. When only the two first conditions are present, the term moyamoya synd rome is used. The incidence of moyamoya disease is high in the Mongol race, although the moyamoya syndrome is more frequently reported among Caucasians. In the last two decades 41 cases of moyamoya were diagnos ed in two Hungarian and two Scandinavian hospitals, respectively. Thir ty-one patients were operated on-12 unilaterally and 19 bilaterally-ei ther with extracranial/intracranial bypass (29 cases) or with encephal omyosynangiosis (2 cases). After a mean follow-up of seven years, 67.7 % of the operated cases were symptom-free or neurologically improved. The majority of the patients had moyamoya disease and turned out to be of Finno-Ugric or Lapplandish ancestry. These people originally migra ted from the East and belong to the Ural-Altaic family. Our findings s uggest that while the moyamoya syndrome is found in different races, t he moyamoya disease may be limited to people of Eastern (Mongol) origi n. Revascularization surgery may be of benefit to patients with moyamo ya.