gms | German Medical Science

Objective: Previous studies found significantly higher incidence of persistent primitive arteries in Asian moyamoya patients than in the general population. In this study we analyzed the incidence of persistent primitive arteries, demographic data and associated systemic disease of patients with moyamoya phenomenon treated in a single German institution. Our study raises the question whether European moyamoya patients demonstrate a similar prevalence of persistent primitive arteries as in Asian patients.

Method: Two independent readers retrospectively evaluated 122 patients with moyamoya phenomenon. Demographic data and associated systemic disease were abstracted from the case records. Cerebral angiograms were reviewed to determine the occurrence of persistent primitive arteries.

Results: We identified 112 cases with moyamoya disease and 10 with moyamoya syndrome, comparable to other European and American demographic data. Mean age at the time of diagnosis was 38.2 (range 6 to 64 years). Ninety (73.8%) were women, thirty-two (26.2%) were male, associated systemic diseases were found in 4 patients. On cerebral angiogram, 7 cases (5.7%) presented with unilaterally affected vessels, 115 (94.3%) with bilaterally affected vessels. One 14-year-old boy suffering from moyamoya disease presented with a primitive trigeminal artery (0.89%). Additional persistent primitive arteries were not detected among patients with moyamoya disease and moyamoya syndrome.

Conclusions: In contrast to moyamoya patients with Asian descent, European patients do not exhibit a higher prevalence of persistent primitive arteries than the normal population. Consequently, different incidence of persistent primitive arteries seems to represent a uniqueness of Asian moyamoya disease.