Serious and rare, a diagnosis of moyamoya disease can take you by surprise. But, at the AdventHealth Neuroscience Institute, our neurosurgeons specialize in the groundbreaking techniques that help people and their families overcome a diagnosis of this brain disease and the debilitating strokes associated with it. Guided by AdventHealth’s whole-person approach to care, we seek to treat you in mind, body and spirit, and help you return to the life you love.
Moyamoya disease is a progressive and rare cerebrovascular disorder caused by blocked arteries at the base of the brain in the basal ganglia. Primarily affecting children, moyamoya was first discovered and described in Japan. Moyamoya is a Japanese word meaning “puff of smoke,” which describes the look of the tangle of tiny vessels that forms at the site of the blockage.
The first symptom of moyamoya disease is usually a stroke or a mini-stroke (a transient ischemic attack). The strokes are frequently accompanied by muscular weakness or paralysis that affects one side of the body.
Adults may experience similar symptoms from blocked arteries, but usually, they experience a hemorrhagic stroke due to bleeding into the brain.
Other moyamoya symptoms can include:
People who have symptoms of moyamoya should meet with their doctor for a physical exam and a review of their family and medical history. Your doctor may order a combination of tests to determine if moyamoya is the cause of your symptoms or if you have any underlying conditions.
Common moyamoya testing includes:
To generate detailed brain images, MRIs use powerful magnets and radio waves. A doctor may inject dye into a blood vessel to better view your arteries and veins, as well as highlight blood circulation (magnetic resonance angiography). Your doctor may also recommend a perfusion MRI, which measures the amount of blood passing through the vessels.
Computed Tomography (CT) Scan
A CT scan is a series of X-rays that create detailed images of the brain. As with MRIs, your doctor can inject a dye into a blood vessel to highlight blood flow, which is called a CT angiogram.
During a cerebral angiogram, a doctor inserts a catheter into a blood vessel in the groin and then guides it to the brain using X-ray imaging. A safe dye can be injected through the catheter into the blood vessels of the brain to make them visible to the X-ray imaging.
Doctors use transcranial Doppler ultrasounds to gather information about the blood vessels in the brain using sound waves.
A PET scan provides visual images of brain activity and SPECT scans measure blood flow to the different regions of the brain. A doctor injects a small amount of safe, radioactive material and monitors its presence in the brain with emission detectors.
An EEG monitors the brain’s electrical activity via electrodes that are attached to the scalp. For children with moyamoya symptoms, EEGs are effective at detecting abnormalities.
With moyamoya disease, the arteries in the affected area of the brain progressively narrow, leading to multiple strokes, mental decline and fatal intracerebral hemorrhage. While medications can help reduce the risk of stroke, surgery is the recommended treatment for patients with moyamoya disease.
At the AdventHealth Neuroscience Institute, our surgeons specialize in advanced surgical techniques to restore blood flow to the brain. These procedures either focus on opening the narrowed blood vessels or bypassing blocked arteries. For some people, this eliminates moyamoya strokes and their related conditions.
The two types of surgical approaches for patients with moyamoya are direct and indirect revascularization. Typically, children respond better to revascularization surgery than adults.
With direct revascularization, surgeons work on the outer surface of the brain, connecting a branch of a scalp artery to a branch of the brain artery to provide immediate improvement in the blood supply to the patient’s brain.
These procedures work to provide more blood flow to the brain. These include:
A branch of the temporal artery is laid directly on the surface of the brain without making a direct connection. This forms a new blood supply.
The temporalis muscle is dissected and then placed on the surface of the brain in this indirect bypass operation. A new blood supply develops from the transposed muscle.
This surgical procedure lays the blood-rich lining surrounding the organs in the abdomen onto the surface of the brain, allowing new blood vessels to develop, grow and flow into the brain.
For most patients, AdventHealth’s moyamoya procedures require an average of three days of hospitalization. There is usually post-operative pain at the incision site on the scalp and some patients experience headaches during their recovery. But, once released, most moyamoya patients can return to their everyday lives with minimal restrictions after surgery.
Moyamoya affects people of all ages. What they each have in common is the need for attentive and responsive care, without delays. Here, patients work with a personal Care Coordinator who guides them through the process, ensuring that their journey through treatment ends with the best possible outcome for their health.