Neuroscience Institute Offers Microsurgical Treatment for Moyamoya Disease

CINCINNATI—The Neuroscience Institute at the University of Cincinnati (UC) and University Hospital is emerging as an important treatment center for patients suffering from moyamoya disease.

Moyamoya disease, a rare brain disorder, is characterized by a chronic and progressive narrowing of the carotid arteries where they enter the brain, causing debilitating strokes and seizures in children and adults.

Mario Zuccarello, MD, a neurosurgeon with the Neuroscience Institute and the Mayfield Clinic, has surgically treated 25 patients with moyamoya during the last five years.

The treatment, which is not widely offered, involves surgically bypassing the blocked arteries. Zuccarello has performed the most delicate and technically challenging bypass procedure, called an extracranial-intracranial bypass. During the procedure, the surgeon connects the superficial temporal artery from the scalp outside the skull to the middle cerebral artery inside the skull.

“We are seeing more patients with moyamoya because patients and physicians are starting to recognize the disease more frequently and because the treatment, which requires highly specialized microvascular techniques, is available here at the Neuroscience Institute,” says Zuccarello, professor of neurosurgery and director of the division of cerebrovascular surgery at the UC College of Medicine.

Although moyamoya disease is most prevalent in Japan, where it was first described, it has been diagnosed in people throughout the world. In the United States, the disorder affects less than one in 100,000 people. However, the condition is associated with six percent of childhood strokes. Because moyamoya disease runs in families, doctors believe the disorder has a genetic cause.

In children, symptoms of moyamoya are most likely to first appear with a type of stroke known as a transient ischemic attack (TIA), or mini-stroke. During an ischemic stroke, a blockage interrupts the flow of oxygen-carrying blood to the brain. In adults, the disease often appears with a TIA or hemorrhagic (bleeding) stroke.

“Moyamoya should be considered and diagnostic evaluation begun in any patient who experiences symptoms of cerebral ischemia,” says Zuccarello.

Symptoms of ischemia may include episodes of muscle weakness or partial paralysis on one side of the body, speech disturbance, sensory impairment, involuntary movement and visual impairment.

The presence of moyamoya is confirmed by a series of imaging studies that can reveal the characteristic arterial narrowing. To compensate for the narrowing arteries, the brain creates collateral blood vessels in an attempt to facilitate the flow of oxygen-rich blood. These tiny collateral vessels, when seen on a brain scan, have a hazy, filmy appearance—the “puff of smoke” that gives the disorder its Japanese name.

Imaging also can reveal evidence of multiple small strokes.

Doctors usually make a diagnosis of moyamoya if MRI images show diminished blood flow in the internal carotid and middle and anterior cerebral arteries and prominent collateral blood flow at the base of the brain. To confirm the diagnosis of moyamoya syndrome, conventional cerebral angiography is typically required.

The Neuroscience Institute is a regional center of excellence at UC and University Hospital. The center is dedicated to patient care, research, education and the development of new treatments for stroke, brain and spinal tumors, epilepsy, traumatic brain and spinal injury, Alzheimer’s disease, Parkinson’s disease, disorders of the senses (swallowing, voice, hearing, pain, taste and smell) and psychiatric conditions (bipolar disorder, schizophrenia and depression).