Moyamoya disease
Evidence: Unconfirmed
Names
Moya-moya disease
Cerebrovascular moyamoya disease
Progressive intracranial arterial occlusion
Progressive intracranial occlusive arteriopathy
Spontaneous occlusion of the Circle of Willis
Description
Moyamoya is a rare cerebrovascular disease that restricts blood flow to certain arteries in the brain. The lack of blood supply to the brain can result in strokes or seizures. Moreover, to compensate for the reduced blood flow, new networks of smaller vessels develop around the blocked arteries, creating a “puff of smoke” appearance on an angiogram. These smaller vessels are more fragile and prone to developing aneurysms and hemorrhages. Although there is much that remains unknown about the disease, it is believed that there is an autoimmune component because of the significantly higher prevalence of autoimmune diseases among Moyamoya patients.
“Moyamoya” is a Japanese word that means “puff of smoke,” which was used to describe the appearance of the collateral vessels that form around blocked arteries. The disease is more common in patients of Asian descent. To treat Moyamoya, physicians may prescribe medications to prevent strokes or perform a surgery called revascularization to improve blood flow in the brain. If patients are left untreated, the disease may result in death.
Patient Groups
Moyamoya Foundation Internet support for those diagnosed with Moyamoya Disease Facebook Group: Moyamoya families Facebook Group: World Moyamoya day Facebook Group: Moyamoya Disease (Rare Brain Disease)
Bloggers
Prevalence
US Incidence: 1/1,100,000 [Orphanet 2015]
Typical Age of onset
Young children (5-9 years) and adults (mid-40s)
Symptoms
stroke
recurrent transient ischemic attacks (TIA, commonly referred to as “mini-strokes”)
muscular weakness
muscular paralysis affecting one side of the body
headaches
seizures
disturbed consciousness
involuntary movements
vision problems
cognitive and/or sensory impairment
References
Profile by Bonnie Shea
Updated Nov. 2022