Moyamoya Disease

Moyamoya disease is a rare, progressive cerebrovascular disorder caused by the narrowing or blockage of the internal carotid artery and its branches, leading to a reduction in blood flow to the brain.

Causes and Risk Factors

The exact cause of Moyamoya disease is unknown, but it is believed to be related to a combination of genetic and environmental factors. Risk factors include:

• Family history: Moyamoya disease can run in families, suggesting a possible genetic component.
• Asian ancestry: The disease is more common in people of Asian descent, particularly Japanese and Korean populations.
• Female sex: Women are more likely to develop Moyamoya disease than men.
• Age: The disease can occur at any age, but it typically affects children and young adults.

Symptoms

The symptoms of Moyamoya disease can vary depending on the severity of the condition and the location of the narrowed or blocked arteries. Common symptoms include:

• Recurring strokes or transient ischemic attacks (TIAs)
• Seizures
• Headaches
• Dizziness or vertigo
• Weakness or numbness in the face, arm, or leg
• Difficulty with speech or language
• Cognitive impairment

Diagnosis

Moyamoya disease is typically diagnosed using a combination of imaging tests, including:

• Magnetic resonance imaging (MRI)
• Magnetic resonance angiography (MRA)
• Computed tomography (CT) scans
• Angiography

Treatment

Treatment for Moyamoya disease usually involves a combination of medical and surgical interventions. Medical treatment may include:

• Antiplatelet agents to prevent blood clots
• Anticoagulants to prevent stroke
• Pain management medications

Surgical options may include:

• Direct bypass surgery: This involves creating a new pathway for blood flow by connecting a healthy artery to the affected area.
• Indirect bypass surgery: This involves promoting the growth of new blood vessels to compensate for the blocked or narrowed arteries.

Prognosis

The prognosis for Moyamoya disease varies depending on the severity of the condition and the effectiveness of treatment. With proper medical and surgical management, many people with Moyamoya disease can experience significant improvement in their symptoms and quality of life. However, the disease can be progressive, and some individuals may experience ongoing cognitive decline or recurrent strokes.