Moyamoya disease is an extremely rare disorder in most parts of the world except in Japan. The pathogenesis of Moyamoya disease is unknown. Moyamoya disease is characterized by progressive intracranial vascular stenoses of the circle of Willis, resulting in successive ischemic events. Hemmorhagic events can also occur. The condition leads to irreversible blockage of the main blood vessels-the carotid arteries- to the brain as they enter into the skull. It is a disease that tends to affect children and adults in the third to fourth decades of life. In children it tends to cause strokes or seizures. In adults it tends to cause bleeding or strokes. The clinical features are cerebral ischaemia (strokes), recurrent transient ischaemic attacks (TIA's or "mini strokes"), sensorimotor paralysis (numbness in the extremeties), convulsions and/or migraine-like headaches.

The process of blockage (vascular occlusion) once it begins tends to continue despite any known medical management unless treated with surgery. The repeated strokes can lead to severe functional impairment or even death so it is important to recognize these lesions and treat them early on. Without treatment, there is progressive deterioration of neurologic function and re-hemorrhage.

The diagnosis is initially suggested by CT, MRI, or angiogram. Contrast-enhanced T1-weighted images are better than FLAIR images for depicting the leptomeningeal ivy sign in moyamoya disease. MRI and MRA should be performed for the diagnosis and follow-up of moyamoya disease. Diffusion-weighted imaging can also be used for following the clinical course of children with moyamoya disease, in whom new focal deficits are highly suspicious of new infarcts.

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