Moyamoya Disease
Moyamoya disease is a rare, progressive cerebrovascular disorder caused by blocked arteries at the base of the brain in an area called the basal ganglia. The name “moyamoya” means “puff of smoke” in Japanese and describes the look of the tangle of tiny vessels formed to compensate for the blockage. Moyamoya disease was first described in Japan in the 1960’s and it has since been found in individuals in the United States, Europe, Australia, and Africa. The disease primarily affects children, but it can also occur in adults. In children, the first symptom of Moyamoya disease is often stroke, or recurrent transient ischemic attacks (TIA, commonly referred to as “mini-strokes”), frequently accompanied by muscular weakness or paralysis affecting one side of the body, or seizures. Adults most often experience a hemorrhagic stroke due to recurring blood clots in the affected brain vessels. Individuals with this disorder may have disturbed consciousness, speech deficits (usually aphasia), sensory and cognitive impairments, involuntary movements, and vision problems. Because it tends to run in families, researchers think that Moyamoya disease is the result of inherited genetic abnormalities. Studies that look for the abnormal gene(s) may help reveal the biomechanisms that cause the disorder.
Treatment
There are several types of revascularization surgery that can restore blood flow to the brain by opening narrowed blood vessels or by bypassing blocked arteries. Children usually respond better to revascularization surgery than adults, but the majority of individuals have no further strokes or related problems after surgery.
Prognosis
Without surgery, the majority of individuals with Moyamoya disease will experience mental decline and multiple strokes because of the progressive narrowing of arteries. Without treatment, Moyamoya disease can be fatal as the result of intracerebral hemorrhage.
Research
The NINDS conducts neurological research in laboratories at the NIH and also supports additional research through grants to major medical institutions across the country. These studies, which range from clinical trials to investigations of basic biological mechanisms, are aimed at discovering how and why diseases develop in the brain, and focus on finding ways to prevent, treat, or cure them.
Organizations
National Rehabilitation Information Center (NARIC)
8400 Corporate Drive
Suite 500
Landover, MD 20785
naricinfo@heitechservices.com
http://www.naric.com
Tel: Landover
Fax: 301-562-2401
National Organization for Rare Disorders (NORD)
Federation of voluntary health organizations dedicated to helping people with rare "orphan" diseases and assisting the organizations that serve them. Committed to the identification, treatment, and cure of rare disorders through programs of education, advocacy, research, and service.
55 Kenosia Avenue
Danbury, CT 06810
orphan@rarediseases.org
http://www.rarediseases.org
Tel: Danbury
Fax: 203-798-2291
National Stroke Association
National non-profit organization that offers education, services and community-based activities in prevention, treatment, rehabilitation and recovery. Serves the public and professional communities, people at risk, patients and their health care providers, stroke survivors, and their families and caregivers.
9707 East Easter Lane
Suite B
Centennial, CO 80112-3747
info@stroke.org
http://www.stroke.org
Tel: Centennial
Fax: 303-649-1328
Children's Hemiplegia and Stroke Assocn. (CHASA)
CHASA is a 501(c)(3) nonprofit organization dedicated to improving the lives of children and families affected by pediatric stroke and other causes of hemiplegia. Offers national family retreat, local family events and seminars, online support group, websites, fact sheets, clinical study information, and pediatric stroke awareness campaigns.
4101 West Green Oaks Blvd., Ste. 305
PMB 149
Arlington, TX 76016
info437@chasa.org
http://www.chasa.org
Tel: Arlington
Content Provided By
NINDS Disorders is an index of neurological conditions provided by the National Institute of Neurological Disorders and Stroke. This valuable tool offers detailed descriptions, facts on treatment and prognosis, and patient organization contact information for over 500 identified neurological disorders.
