Moyamoya disease

Moyamoya disease is a rare, progressive cerebrovascular disorder characterized by the narrowing or blockage of arteries at the base of the brain, specifically the internal carotid arteries and their main branches.

The term 'moyamoya' is Japanese for 'puff of smoke', which describes the appearance of the tangled, small blood vessels that develop to compensate for the blocked arteries.

These collateral vessels are fragile and prone to bleeding, leading to an increased risk of stroke.

Moyamoya disease affects the brain by reducing blood flow, which can result in transient ischemic attacks (TIAs), strokes, and hemorrhages.

Symptoms may include headaches, seizures, and cognitive or developmental delays, particularly in children.

Diagnosis typically involves imaging studies such as MRI and cerebral angiography to visualize the characteristic vascular changes.

Early diagnosis and treatment, which may involve surgical revascularization procedures to restore blood flow, are crucial to managing the condition and preventing further neurological damage.

This complex condition requires a multidisciplinary approach to care, involving neurologists, neurosurgeons, and other specialists to optimize outcomes for affected individuals.