What is moye moye?

Moyamoya Disease

Moyamoya disease is a rare, progressive cerebrovascular disorder caused by stenosis or occlusion of the arteries in the circle of Willis at the base of the brain. The word "moyamoya" means "puff of smoke" in Japanese, describing the appearance of the tiny clusters of blood vessels that form to compensate for the blocked arteries.

Causes: The exact cause of moyamoya disease is unknown, but it is believed to have a genetic component. It's more common in people of East Asian descent, particularly Japanese, Korean, and Chinese individuals. Certain conditions, such as Down syndrome, sickle cell anemia, and neurofibromatosis type 1, are associated with an increased risk of developing moyamoya.

Symptoms:

Symptoms vary depending on age and the severity of the condition.

• In children, the most common symptom is stroke or transient ischemic attack (TIA), which can cause weakness or paralysis on one side of the body, speech difficulties, seizures, and developmental delays. Headaches are also common.
• In adults, symptoms may include hemorrhagic stroke (bleeding in the brain), headaches, seizures, and cognitive decline. TIAs are also possible.

Diagnosis:

Diagnosis typically involves a combination of:

• Medical history and physical exam: Assessing symptoms and risk factors.
• Neuroimaging:
  • Magnetic Resonance Imaging (MRI) and Magnetic Resonance Angiography (MRA): To visualize the brain and blood vessels.
  • Cerebral Angiography (Arteriography): A more invasive procedure that provides detailed images of the arteries in the brain.
  • Computed Tomography (CT) Scan: Useful in identifying bleeding within the brain, particularly in acute situations.
  • Transcranial Doppler Ultrasound (TCD): To measure blood flow velocity in the brain's arteries.

Treatment:

The goal of treatment is to prevent strokes and improve blood flow to the brain.

• Medications: Aspirin or other antiplatelet drugs may be prescribed to reduce the risk of blood clots.
• Surgical revascularization: Surgical procedures aim to bypass the blocked arteries and restore blood flow. Common procedures include:
  • Direct bypass: Connecting a scalp artery directly to a brain artery (e.g., superficial temporal artery-middle cerebral artery (STA-MCA) bypass).
  • Indirect bypass: Placing tissue on the surface of the brain to encourage new blood vessel growth (e.g., encephaloduroarteriosynangiosis (EDAS), encephalomyosynangiosis (EMS)).
  • Combined approaches: Using both direct and indirect bypass techniques.

Prognosis:

The prognosis varies depending on the severity of the disease, the age of onset, and the effectiveness of treatment. Early diagnosis and treatment can significantly improve outcomes. Without treatment, moyamoya disease can lead to severe neurological deficits, stroke, and even death.