Moyamoya Disease in a Turkish Woman: A Case Illustration

Author(s): Mehtap Erdogan*, Keziban Karacan, Alper Karacan, Ayla Tekin Orha, Tuncay Colak and Hamit Macit Selekler

The etiology of Moyamoya Disease (MMD) is unknown. It is a cerebrovascular disease which is caused by occlusion or stenosis and can be diagnosed by angiographic methods. Digital Subtraction Angiography (DSA) is the gold standard for diagnosis, especially in cases with mild stenosis and few collateral lesions. The causes of this disease are unknown; it is usually associated with congenital or tumoral defects. This disease is more common in the yellow race, especially woman in Japan. Its prevalence is low in countries other than Japan. The high prevalence of the disease in Japan has given it its name from Japanese. Moyamoya; Meaning of Japanese means smoke dispersing in the air.

The veins in the appearance of smoke scattering in the air giving the name of Moyamoya are, in fact, perforating arteries which are enlarged and angiographically visible to form collateral pathways due to stenosis. The first symptoms in Moyamoya may occur in various forms, including recurrent headache, epilepsy, acute hemiplegia. The clinical picture varies between children and adults. Ischemia in children and early hemorrhage in adults. Angiography is accepted as the gold standard for diagnosis of MMD. The criteria for angiographic diagnosis of MMD have been determined by the Research Committee for Progressive Obstructive Diseases of Circle of Willis of the Ministry of Health of Japan.

In this article, a 38-year-old female patient, who was admitted to the emergency department with complaints of severe headache and vomiting two years ago and whose hemorrhage areas were detected and discharged with conservative treatment, was admitted to the emergency department with sudden onset hemiparesis, nausea, vomiting and left hemiparesis. We present a case of MMD diagnosed in a 38-yearold female patient presenting with right capsula interna crus posterior and basal core hemorrhage on Magnetic Resonance Angiography (MRA) images.


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