Moyamoya disease pathophysiology: Difference between revisions
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==Pathophysiology== | ==Pathophysiology== | ||
The constrictions of the arteries in moyamoya disease are unlike the constrictions in [[atherosclerosis]]. In atherosclerosis, the inner layer ([[Lumen (anatomy)|lumen]]) of the arteries suffers an immune reaction, fills with inflammatory cells, and accumulates fatty cells and debris. In moyamoya, the inner layer of the carotid artery overgrows inward to constrict the artery, and the artery also fills with blood clots, which cause strokes.<ref name="Scott">[http://content.nejm.org/cgi/content/full/360/12/1226 Moyamoya Disease and Moyamoya Syndrome], R. Michael Scott and Edward R. Smith, New England Journal of Medicine, 360:1226-1237, March 19, 2009</ref> | The constrictions of the arteries in moyamoya disease are unlike the constrictions in [[atherosclerosis]]. In atherosclerosis, the inner layer ([[Lumen (anatomy)|lumen]]) of the arteries suffers an immune reaction, fills with inflammatory cells, and accumulates fatty cells and debris. In moyamoya, the inner layer of the carotid artery overgrows inward to constrict the artery, and the artery also fills with blood clots, which cause strokes.<ref name="Scott">[http://content.nejm.org/cgi/content/full/360/12/1226 Moyamoya Disease and Moyamoya Syndrome], R. Michael Scott and Edward R. Smith, New England Journal of Medicine, 360:1226-1237, March 19, 2009</ref> | ||
The condition is believed to be hereditary and linked to q25.3, on chromosome 17 [http://emedicine.medscape.com/article/1180952-overview#ClinicalCauses]. They have a higher risk of recurrent stroke and may be experiencing a distinct underlying pathophysiology compared to patients from Japan. Data suggest a potential benefit with surgery if early diagnosis is made.<ref name="pmid16645133">{{cite journal |author=Hallemeier C, Rich K, Brubb R, Chicoine M, Moran C, Cross D, Zipfel G, Dacey R, Derdeyn |title=Epidemiological features of moyamoya disease in Japan: findings from a nationwide survey |journal=[[Stroke]] |volume=37 |issue= 6|pages=1490–1496 |year=2006 |pmid=16645133 |doi=10.1161/01.STR.0000221787.70503.ca}}</ref> The pathogenesis of moyamoya disease is unknown. | |||
Once it begins, the process of blockage (vascular occlusion) tends to continue despite any known medical management. In some people this leads to repeated strokes and severe functional impairment or even death. In others, this blockage may not cause any symptoms. | |||
Moyamoya can be either congenital or acquired. Patients with [[Down syndrome]], [[neurofibromatosis]], or [[sickle cell disease]] can develop moyamoya malformations. It is more common in women than in men, although about a third of those affected are male .<ref name="pmid18048855">{{cite journal |author=Kuriyama S, Kusaka Y, Fujimura M, ''et al.'' |title=Prevalence and clinicoepidemiological features of moyamoya disease in Japan: findings from a nationwide epidemiological survey |journal=Stroke |volume=39 |issue=1 |pages=42–7 |year=2008 |pmid=18048855 |doi=10.1161/STROKEAHA.107.490714 |url=http://stroke.ahajournals.org/cgi/pmidlookup?view=long&pmid=18048855}}</ref> Brain radiation therapy in children with neurofibromatosis increases the risk of its development. | |||
==References== | ==References== | ||
{{reflist|2}} | {{reflist|2}} | ||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Vishnu Vardhan Serla M.B.B.S. [2]
Pathophysiology
The constrictions of the arteries in moyamoya disease are unlike the constrictions in atherosclerosis. In atherosclerosis, the inner layer (lumen) of the arteries suffers an immune reaction, fills with inflammatory cells, and accumulates fatty cells and debris. In moyamoya, the inner layer of the carotid artery overgrows inward to constrict the artery, and the artery also fills with blood clots, which cause strokes.[1]
The condition is believed to be hereditary and linked to q25.3, on chromosome 17 [3]. They have a higher risk of recurrent stroke and may be experiencing a distinct underlying pathophysiology compared to patients from Japan. Data suggest a potential benefit with surgery if early diagnosis is made.[2] The pathogenesis of moyamoya disease is unknown.
Once it begins, the process of blockage (vascular occlusion) tends to continue despite any known medical management. In some people this leads to repeated strokes and severe functional impairment or even death. In others, this blockage may not cause any symptoms.
Moyamoya can be either congenital or acquired. Patients with Down syndrome, neurofibromatosis, or sickle cell disease can develop moyamoya malformations. It is more common in women than in men, although about a third of those affected are male .[3] Brain radiation therapy in children with neurofibromatosis increases the risk of its development.
References
- ↑ Moyamoya Disease and Moyamoya Syndrome, R. Michael Scott and Edward R. Smith, New England Journal of Medicine, 360:1226-1237, March 19, 2009
- ↑ Hallemeier C, Rich K, Brubb R, Chicoine M, Moran C, Cross D, Zipfel G, Dacey R, Derdeyn (2006). "Epidemiological features of moyamoya disease in Japan: findings from a nationwide survey". Stroke. 37 (6): 1490–1496. doi:10.1161/01.STR.0000221787.70503.ca. PMID 16645133.
- ↑ Kuriyama S, Kusaka Y, Fujimura M; et al. (2008). "Prevalence and clinicoepidemiological features of moyamoya disease in Japan: findings from a nationwide epidemiological survey". Stroke. 39 (1): 42–7. doi:10.1161/STROKEAHA.107.490714. PMID 18048855.