De novo development of moyamoya disease in an adult female

The origin of moyamoya disease remains unknown. The onset of the angiographically apparent changes of typical moyamoya disease occurs in childhood, but de novo development of the disease has not been confirmed angiographically. The authors report on a case of de novo development of moyamoya disease in a middle-aged female whose cerebral angiography demonstrated no abnormal findings 5 years previously. To the best of the authors' knowledge, this case is the first reported instance of de novo development of definite moyamoya disease verified angiographically. This case demonstrates that the de novo development of moyamoya disease in a middle-aged adult did in fact occur, and angiographically visible features of the disease took < 5 years to complete. (DOI: 10.3171/2009.2.JNS081244)