Moyamoya Disease

Moyamoya disease is a rare condition that affects the blood vessels in the brain, reducing blood flow and increasing the risk of stroke. With this disease, the walls of the arteries in the brain become thickened and narrowed. The body tries to compensate by creating new blood vessels, but these are often fragile and prone to bleeding

The name “moyamoya” means “puff of smoke” in Japanese, describing the tangled appearance of these small vessels on imaging scans. 

At Northeast Georgia Health System our neurosurgery and neurointerventional teams specialize in diagnosing and treating Moyamoya disease with advanced imaging and surgical techniques, including Moyamoya surgery to restore blood flow to the brain. 

What causes Moyamoya disease?

The exact cause of Moyamoya disease is unknown, but several risk factors increase the likelihood of developing it: 

  • Genetics: A person has a 50% chance of inheriting the mutated gene that causes Moyamoya disease from an affected parent. People with a family history of Moyamoya disease are at higher risk of developing the condition.
  • Ethnicity: Moyamoya disease is more common in people of Asian descent, particularly in individuals of Japanese or Korean ancestry.
  • Age: Moyamoya disease is diagnosed most in children and young adults under the age of 40, although it can occur at any age.
  • Medical history: Certain medical conditions, such as sickle cell anemia, neurofibromatosis type 1, and other autoimmune or inflammatory conditions, may increase the risk of developing Moyamoya disease.
  • Radiation therapy: Exposure to radiation therapy to the brain, particularly in childhood, has been associated with an increased risk of Moyamoya disease.
What are the symptoms of Moyamoya disease?

Some common symptoms of Moyamoya disease include:

  • Recurrent strokes or TIAs
  • Seizures
  • Cognitive impairment and memory loss
  • Headache
  • Weakness or numbness in the arms or legs
  • Vision problems, including double vision, loss of peripheral vision, or blindness in one eye.

If you or a loved one experience these symptoms, seek medical attention immediately, as moyamoya can lead to life-threatening complications without treatment. 

How do you diagnose Moyamoya disease?

Our specialists use advanced imaging and testing to confirm moyamoya disease: 

  • MRI or CT scan: Identifies brain changes or prior strokes. 
  • MRA or CTA: Uses contrast dye to highlight narrowed or blocked arteries. 
  • Cerebral angiogram: Provides the most detailed images of brain blood vessels. 
  • Transcranial Doppler ultrasound: Measures blood flow through arteries. 
  • Neuropsychological testing: Assesses memory, thinking and cognitive function. 

Moyamoya Surgery & Treatment

The treatment of Moyamoya disease typically involves a combination of medical management, lifestyle changes and surgical intervention. The goal of treatment is to improve blood flow to the brain, prevent strokes, and manage any complications or associated medical conditions.

Medical management

Treatment of Moyamoya disease may involve managing risk factors for stroke, such as high blood pressure, high cholesterol, and diabetes.

  • Blood-thinning medications (e.g., aspirin) to prevent clots 
  • Blood pressure, cholesterol and diabetes management 
  • Lifestyle support, including diet and exercise 

Surgical intervention

The primary surgical treatment for moyamoya is revascularization, which creates new pathways for blood flow: 

  • Direct bypass surgery: Connects a healthy scalp artery directly to a brain artery. 
  • Indirect bypass surgery: Encourages new blood vessels to grow into the affected area over time. 

At NGHS our neurosurgery and neurointerventional surgery program offers both minimally invasive endovascular approaches and open surgical options when needed. These advanced techniques often eliminate the need for traditional open surgery and can be lifesaving for patients once considered untreatable. 

Why choose NGHS for Moyamoya care

  • Board-certified neurosurgeons and neurointerventional specialists 
  • State-of-the-art imaging and surgical technology 

Find expert care for Moyamoya disease

Living with moyamoya disease can feel overwhelming, but you don’t have to face it alone. At Northeast Georgia Health System our team of specialists is here to guide you from diagnosis through treatment, offering the latest options in moyamoya surgery and ongoing support for your recovery.

To get started, you will need a referral from your primary care provider or another clinician. If you have not yet received a diagnosis but are experiencing symptoms of moyamoya, you may want to begin by scheduling an appointment with one of our neurologists.