Moyamoya Disease: A Review of Literature

Abstract

Moyamoya disease is a rare progressive cerebrovascular disorder caused by blocked Supraclinoid Internal Carotid Arteries at the base of the brain in the basal ganglia area. It is a chronic and progressive condition. The word “Moyamoya” is Japanese which refers to a hazy puff of smoke or cloud due to the appearance of blood vessels like a puff of smoke in angiograms of people suffering from this disease. The exact etiology of Moyamoya disease remains unclear, but there seems to be genetic and acquired forms. The first sign of Moyamoya disease is usually stroke or recurrent Transient Ischemic Attacks (TIAs) also called “mini-strokes”. Some other symptoms may include brain hemorrhage, headaches, developmental delays, aneurysm, involuntary movements, problems with cognitive abilities, problems with the senses, seizures, hemiparesis, ischaemic stroke or hemorrhagic stroke. Despite the etiology of Moyamoya disease being unclear, there are certain factors which may increase the risk of getting the disease, like Asian ancestry, family history of Moyamoya disease, other medical conditions, being female and being young. Moyamoya disease has a very unclear etiology and pathogenesis. Moyamoya disease can be diagnosed using tests like Cerebral Arteriography, Magnetic Resonance Angiography (MRA), Magnetic Resonance Imaging (MRI), etc. There is no cure for Moyamoya disease, but can be treated using drugs and surgical procedures. Hence, extensive studies need to be conducted in order to better determine the exact pathophysiology of the disease and also to find more effective treatment options that would further improve the prognosis in patients with Moyamoya Disease.