Moyamoya disease(모야모야병)

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목차

1. EPIDEMIOLOGY
2. PATHOGENESIS AND ETIOLOGY
3. PATHOLOGY
4. CLINICAL PRESENTATION
5. DIAGNOSTIC EVALUATION
6. TREATMENT
7. OUTCOMES
8. MOYAMOYA DISEASE AND PREGNANCY

본문내용

- Disease ; unknown etiology
Syndrome ; poor intracranial perfusion caused by Known disorder
Ex) arteriosclerosis
- Chronic progressive stenosis of the arteries of the circle of Willis.
- Initial manifestation ;
stenosis of the arteries of bilateral supraclinoid carotid arteries,
development of abnormal collateral circulation at the base of the brain and
basal ganglia
- Progress to involve the MCA and posterior cerebral arteries
- Lead to ischemic strokes or spontaneous intracranial hemorrhages

1. EPIDEMIOLOGY
- Occur worldwide
Highest in Japan, China, Korea
Higher in woman (1.7:1)
- Age onset : bimodal
Younger than age 10 years ; ischemic disorder
Fourth decade ; hemorrhage
- Incidence of familial subtype ; 8.82%

2. PATHOGENESIS AND ETIOLOGY
- Remain obscure
- Delayed repair mechanism for vascular wall
slow but long term proliferation of smooth muscle during repair process
--> Progressive occlusion of intracranial vessels
- Role of angiogenic growth factors - on investigation
b-FGF ; possible mediator of the neovascular response in moyamoya Dz.
- Human leukocyte antigen (HLA) class 2 genes

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