Moya-Moya Disease a Case Report and Literature Review

Abstract

Moya-Moya disease is a rare chronic cerebral vascular disease (arteriopathy), which can affect both children and adults and gives rise to severe clinical manifestations of ischemic or hemorrhagic origin. This angiogenic disease is an intrinsic primary pathological process, characterized by a narrowing of the distal internal carotid artery that extends to the proximal segments of the middle and anterior cerebral arteries, inducing the formation of replacement vessels that give a characteristic appearance on angiography forming a smoke cloud: Moya Moya network. The involvement of the posterior circulation is very rare. Its etiology is still poorly understood and represents 10 to 15% of the causes of strokes, with 2 age peaks where it is more frequent: children around 5 years and adults around 40 years. Its evolution can be slow with intermittent symptoms or fulminant with a rapid neurological decline. Imaging is the reference examination in the diagnosis of Moya-Moya. MRI (Magnetic Resonance Imaging) has an important and growing role in the management of the disease. We report a case of a 48 year old patient with diabetes and hypertension, hospitalized for a deep left hematoma.