Moyamoya Disease

Description

Moyamoya Disease »

Moyamoya disease is characterized by chronic and progressive narrowing internal carotid arteries in the brain that can lead to complete blockage. To compensate for the narrowing arteries, the brain creates collateral blood vessels at the base of the brain in an attempt to facilitate the flow of oxygen-rich blood. The Japanese were the first to describe the condition. They named it “moyamoya,” the Japanese term for “puff of smoke,” because of the blood vessels’ hazy, filmy appearance on an angiography scan.

Most prevalent in Japan, fewer than one in 100,000 people in the U.S. are at risk of developing the disease. In adults, the disease can first appear with an ischemic stroke or transient ischemic attack (TIA), a blockage that interrupts the flow of oxygen-carrying blood to the brain, or with a hemorrhagic (bleeding) stroke.

Signs and Symptoms

Signs and symptoms of moyamoya are similar to those of ischemic stroke, hemorrhagic stroke or TIA and include: muscle weakness or partial paralysis on one side of the body, speech disturbance, sensory impairment, involuntary movement, and/or visual impairment.

Diagnosis

A brain MRI is used determine the patient’s stroke history, followed by a CT angiogram or conventional cerebral angiogram to identify the characteristic arterial narrowing and collateral blood vessels associated with moyamoya disease.

Treatment

There is no known medication that can reverse the progression of moyamoya disease. Surgery is generally recommended to treat patients with recurrent or progressive ischemic strokes or TIAs and involves directly connecting a donor artery to a recipient artery in the brain.

Revascularization through bypass surgery prevents further brain injury by using the external carotid circulation to increase collateral blood flow to underserved areas of the brain. Often, blood vessels are directly connected by a bypass to link the superficial temporal artery (STA) to the middle cerebral artery.

  • Print This Page
  • Make an Appointment: Schedule Now
  • FY 2015 Annual Report

  • Everyone is at risk for a stroke. What's your risk level?
  • UCNI Weekly Blog
  • Hope Stories

    • Christine’s Story: Stroke

      Christine's Story: StrokeThere was a sliver of a chance, maybe, and most likely the emergency room doctor thought there was no chance at all. Christine had arrived at the community hospital comatose, brought by her parents, who had come home after working...
    • Jeff’s Story: Ruptured Aneurysm, Airway Reconstruction

      Jeff's Story: Ruptured Aneurysm, Airway Reconstruction Jeff’s remarkable story has two parts: recovery and reconstruction. He doesn’t remember the first part -- the recovery from a ruptured aneurysm. But he vividly remembers the second part -- the reconstruction of his airway. He is living the followup to...
    • Richard’s Story: Ruptured Aneurysms

      Richard's Story: Ruptured Aneurysms Almost three years ago, Richard “Dick” Watson, MD, found himself in an unfamiliar position for a doctor: lying on the operating table instead of standing over it. He didn’t know it at the time, but it was the beginning of...
    • Marlene’s Story: Facebook was her Friend

      Marlene's Story: Facebook was her Friend In treating a stroke, time is brain. Because treatment must be administered within 3 to 4 ½ hours to be eff ective, one of the first things a doctor or EMT must do is determine the moment when the stroke...
    • Zettie’s Story: Aphasia

      Zettie's Story: Aphasia In November of 2004, Zettie Williams confronted what neurologists consider one of the most feared consequences of stroke. When a therapist showed her a photograph of her son, she knew she was looking at her son, but she couldn’t say...