- published: 06 Feb 2014
- views: 7218
Moyamoya syndrome is a disease in which certain arteries in the brain are constricted. Blood flow is blocked by the constriction, and also by blood clots (thrombosis).
A collateral circulation develops around the blocked vessels to compensate for the blockage, but the collateral vessels are small, weak, and prone to hemorrhage, aneurysm and thrombosis. On conventional X-ray angiography, these collateral vessels have the appearance of a "puff of smoke" (described as "もやもや (moyamoya)" in Japanese).
About 10% of cases of moyamoya disease are familial, and some cases result from specific genetic mutations. Susceptibility to moyamoya disease-2 (MYMY2; 607151) is caused by variation in the RNF213 gene (613768) on chromosome 17q25. Moyamoya disease-5 (MYMY5; 614042) is caused by mutation in the ACTA2 gene (102620) on chromosome 10q23.3; and moyamoya disease-6 with achalasia (MYMY6; 615750) is caused by mutation in the GUCY1A3 gene (139396) on chromosome 4q32. Loci for the disorder have been mapped to chromosome 3p (MYMY1) and chromosome 8q23 (MYMY3; 608796). See also MYMY4 (300845), an X-linked recessive syndromic disorder characterized by moyamoya disease, short stature, hypergonadotropic hypogonadism, and facial dysmorphism. and linked to q25.3, on chromosome 17". (Online Mendelian Inheritance in Man, omim.org/entry/252350).
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Children with moyamoya disease require coordinated care. Treatment includes pial synangiosis surgery, which helps reduce the risk of stroke. http://www.chop.edu/neurosurgery Children with moyamoya disease have narrowing of the carotid arteries, which feed oxygen-rich blood to the brain. This restriction of blood flow to the brain can result in pediatric stroke. Neurosurgeons at The Children’s Hospital of Philadelphia have expertise in pial synangiosis (also called indirect bypass), a surgery for moyamoya disease. In this surgery, an artery from the scalp is rerouted onto the brain and over time grows “roots” — new blood vessels to feed the brain. Moyamoya disease requires care not only from a neurosurgeon, but also from a neurologist. The Pediatric Stroke Program at CHOP, led by pedia...
Fuat Arikan, MD, PhD. Department of Neurosurgery and Neurotraumatology Research Unit (UNINN) Vall d’Hebron University Hospital and Universitat Autònoma de Barcelona
Learn more about the Moyamoya Disease Program: http://www.childrenshospital.org/centers-and-services/moyamoya-disease-program Edward Robert Smith, MD, director of Pediatric Cerebrovascular Surgery at Boston Children's Hospital, illustrates Moyamoya disease and pial synangiosis, an operation developed at Boston Children’s to treat it. Moyamoya is a disease of the blood vessels of the brain that leads to poor blood flow, oxygen starvation and potentially stroke. It is diagnosed by angiogram, on which the diseased vessels look like a “puff of smoke” (the meaning of moyamoya in Japanese). As Dr. Smith explains in our Pediatric Playbook series, pial synangioisis takes a healthy blood vessel from the scalp and grafts it onto the surface of the brain. New blood vessels begin sprouting from it...
Moyamoya is a condition of brain vessel blockages or narrowing that can be treated with various types of bypass procedures. Dr. Achal Achrol at the Pacific Neuroscience Institute, director of the cerebrovascular center performed this operation on Joanna, who was having strokes due to her condition.
Moyamoya disease, which is also known as Moyamoya syndrome, is a rare but very serious condition in which the walls of the internal carotid arteries—the vessels that supply blood to important areas of the brain—become thickened and narrowed. This causes the flow of oxygen-rich blood to the child's brain to gradually slow down, and makes it more likely that a blood clot will form. This reduced blood and formation of blood clots are major risk factors for either a transient ischemic attack (TIA) also called a "mini-stroke,"or a full-fledged stroke. "Moyamoya" means "puff of smoke" in Japanese. The disease gets its name from the wispy, tangled appearance of the new blood vessels that emerge in the brain (as the body attempts to compensate for the inadequate blood supply). Moyamoya disease ...
Moyamoya disease is a condition where the large vessels in the brain narrow over time. As a result, tiny vessels in the brain compensate by getting larger. Visit http://mayocl.in/2ojVQI1 for more information or to request an appointment. As these small vessels get larger, they become stressed and stretched and can rupture leading hemorrhage in the brain which can be disabling and occasionally fatal. Bernard Bendok, M.D., chair of Neurosurgery at Mayo Clinic in Arizona, explains treatment options for the disease that could lead to a better quality of life.
This is the first video I took of Laura after her stroke from Moyamoya. She was not diagnosed with it until three months later after three strokes and a brain bleed. She was misdiagnosed as having Medium vessel vasculitis. She suffered bad headaches which became more frequent up until her stroke. She has since had a cerebral bypass and pial synangiosis surgey. She is recovering well! visit us at http://cartla2.xanga.com/