Moyamoya disease

Symptom of moyamoya disease

In most children, symptoms such as repeated headaches, fainting, cataplexy, and convulsive seizures are present. These symptoms are triggered by specific actions, which may include blowing on hot noodles during eating, playing a musical instrument, or running a race. All of these involve hyperventilation, or repeatedly taking deep breaths over a short time. This is understood to distort the balance of certain substances in the blood, causing the "puff of smoke" blood vessels to constrict and reducing blood flow, resulting in the appearance of symptoms resembling those of cerebral infarction (a form of stroke). This phenomenon whereby blood flow in the "puff of smoke" blood vessels decreases and stroke-like symptoms appear is known as "ischemia". Symptoms of ischemia may alternate between the left and right sides, and may sometimes progress from one side to the other. Further progression of ischemic symptoms may result in the appearance of paralysis or cognitive disability.

Cerebral hemorrhage occurs in around half of adults with moyamoya disease. The other half experience the same ischemic symptoms seen in children. The symptoms of cerebral hemorrhage are sudden onset of headache, impaired consciousness, and paralysis. Emergency treatment is required. Hemorrhage is believed to occur when the walls of the "puff of smoke" blood vessels become weakened or form a bulge.

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