Moyamoya Disease

Dr. Ulm and Dr. Mericle are two of the most experienced neurovascular surgeons in Tennessee. Together they have performed dozens of brain bypass operations (EC-IC bypass) and EDAS revascularization procedures. Prior to re-locating to Baptist Hospital in Nashville, Dr. Ulm founded the Moya-Moya clinic at the Medical Center of Central Georgia and re-started the bypass program at LSU in New Orleans. Dr. Mericle performed the vast majority of all EC-IC bypasses done at Vanderbilt University Medical Center and Childrens hospital over the past decade.

Moyamoya disease is a progressive disease of the proximal cerebral blood vessels. It is characterized by narrowing of the internal carotid artery, middle cerebral artery, and anterior cerebral artery. The condition leads to irreversible blockage of the main blood vessels to the brain as they enter into the skull.

Moyamoya tends to affect children, as well as adults in the third to fourth decades of life. Children present with symptoms of stroke, such as weakness of an arm or leg, or seizures. Adults tend to present with ischemic or hemorrhagic stroke.

Signs & Symptoms

Patients tend to present with signs of stroke or mini-strokes. They complain of speech difficulties, paralysis of part of the body, weakness, numbness, or loss of consciousness. Symptoms can be permanent or transient.

Diagnosis of Moyamoya

The diagnosis is made by any neuroimaging study that evaluates blood vessels.

MRI with diffusion weighting is the best study to evaluate for evidence of strokes. Often nuclear medicine studies such as SPECT (single photon emission computerized tomography) or a DIAMOX nuclear medicine scan are used to demonstrate the decreased blood and oxygen supply to areas of the brain involved with the Moyamoya disease.

Treatment of Moyamoya Disease

The initial therapy for Moyamoya consists of blood thinning therapy, usually asprin. This helps blood travel through the narrowed intracranial blood vessels. Most patients are followed regularly and have repeat imaging to evaluate any changes in blood vessel size. Surgery is indicated if there are worsening symptoms. An EC-IC bypass provides a direct and immediate supply of fresh blood to the affected area of the brain and is the preferred procedure whenever possible.

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