Moyamoya Disease
Specialties
- Arteriovenous Malformation
- Alzheimer's/Dementia
- Brain Aneurysms
- Brain & Spinal Cord Injuries
- Brain & Spinal Tumors
- Epilepsy/Seizures
- Headaches/Migraines
- Huntington's Disease
- Infectious Diseases
- Movement Disorders
- Moyamoya Disease
- Multiple Sclerosis/ Autoimmune Disorders
- Neuromuscular Disorders
- Parkinson's Disease
- Psychiatry
- Rehabilitation
- Scoliosis
- Stroke
- Trigeminal Neuralgia
IU Health Neuroscience is one of only a few medical centers in the nation with the specialized neurosurgical expertise to provide advanced treatment for Moyamoya disease. Moyamoya disease is a very rare disorder that affects the blood vessels in the brain. The disease causes the blood vessels at the base of the brain to narrow, slowing blood flow to the brain. When blood flow to the brain is reduced or completely blocked, the risk of having a stroke is increased.
Moyamoya disease can affect both children and adults. It affects people of all ethnicities. It is sometimes seen in children with Down Syndrome, neurofibromatosis, sickle cell anemia, Graves’ disease, and in children who have brain tumors near the base of the brain. Some children develop Moyamoya disease after a viral illness. The disorder may run in families, however, in most cases of Moyamoya, there is no known cause.
The disease’s name derives from the Japanese word for “puff of smoke.” When the brain cannot get enough blood, tiny blood vessels form at the base of the brain to try to make up for the lack of blood supply. When seen on a cerebral angiogram—an X-ray that examines how blood flows through the brain—the tangle of new blood vessels can look like a small, dark cloud, or puff of smoke.
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| 1. Symptoms | |
| 2. Diagnosis | |
| 3. Treatment Options | |
| 4. Moyamoya Specialists | |
SYMPTOMS
In many people with Moyamoya disease, including children, the first sign of the disorder is stroke or recurrent transient ischemic attacks (TIAs), which are also called mini-strokes. Parents may notice muscle weakness or paralysis on one side of the child’s body or sudden slurred speech. Other symptoms can include:
- Decline in cognitive functioning or worsening learning impairment
- Seizures
- Severe, persistent headaches
DIAGNOSIS
At IU Health Neuroscience, highly specialized neuroradiologists use sophisticated MRI and CT imaging to examine the blood vessels of the brain. These imaging studies help doctors locate and evaluate the blockage in the carotid arteries at the base of the brain. The studies can also identify signs of past stroke or mini-strokes.
Once the diagnosis of Moyamoya disease is confirmed, neuroradiologists will perform a cerebral angiogram. This detailed study of the arteries helps IU Health neurosurgeons plan treatment. During a cerebral angiogram, a catheter is inserted into the femoral artery and guided up to the carotid artery. A dye is then injected into the artery. X-ray images highlight how the dye moves through the arteries of the brain, helping doctors pinpoint the area and extent of blockage.
TREATMENT OPTIONS
Without treatment, people with Moyamoya disease are at increased risk of stroke, severe disability and death. Medication can help lower the risk for blood clots, but surgery is the only cure for Moyamoya disease.
Pial Synangiosis Surgery
At IU Health Neuroscience, a highly skilled pediatric neurosurgeon performs pial synangiosis surgery for Moyamoya disease. In this delicate procedure, a small incision is made in the scalp to expose a healthy blood vessel. Then, with the aid of a high-powered operating microscope, each one of the brain’s coverings is opened to expose the pial surface of the brain. Using tiny sutures, the scalp artery is secured directly to the pial surface of the brain. Over time, the healthy blood vessel will stimulate the growth of new blood vessels, replacing the lost blood supply from the blocked artery. This process is known as angiogenesis.
While there are other surgical options for Moyamoya disease, pial synangiosis is the most successful treatment option for children. This procedure was introduced at Children’s Hospital of Boston, where Jodi Smith, PhD, MD, a neurosurgeon practicing at IU Health, completed her fellowship training in Pediatric Neurosurgery. She is one of a few neurosurgeons in the nation trained by the surgeon who pioneered this procedure, and she has a near 100 percent success rate performing pial synangiosis.
Because children with Moyamoya disease are at a heightened risk for stroke during surgery, it is especially important that any surgery include anesthesiologists experienced in treating the disorder. Riley Hospital for Children at IU Health has a dedicated Moyamoya anesthesia protocol, and all of the anesthesiologists at Riley at IU Health are experienced in managing Moyamoya disease patients to reduce risk of stroke during surgery.
After surgery, most patients remain in the hospital for about three days and typically return to regular activities in about four weeks. Children can expect to return to school in one to two weeks after surgery.