Moyamoya disease associated with fibromuscular dysplasia of intrapulmonary bronchial arteries-a case report.

A case is reported of a 40-year-old woman clinically diagnosed as moyamoya disease with associated fibromuscular dysplasia of intrapulmonary bronchial arteries incidentally revealed during autoptic examination. Moyamoya disease represents an idiopathic noninflammatory and nonatherosclerotic arterio-occlusive process of intracranial arteries. Prolonged brain ischemia leads to formation of tiny and fragile collaterals. Clinically, patients with moyamoya angiopathy commonly present with severe neurological symptoms caused by brain infarction or hemorrhage. Histologically, the steno-occlusive process is based on fibrocellular thickening of intima and intimal smooth muscle cell proliferation. In the literature, extracranial arterial involvement, i.e. fibromuscular dysplasia of renal or pulmonary arteries, has been described in several cases of moyamoya disease. Our aim is to show a unique case of moyamoya disease associated with fibromuscular dysplasia affecting an uncommon site.