Liang Ziyang  Wei Shihui  Zhang Maonian

Ophthalmology department of General Hospital of PLA， Beijing China100853

Objective： To probe the clinic characteristics and mechanisms of ocular onset features in patients with Moyamoya disease. Methods： A retrospective analysis was performed on 74 patients who had Moyamoya disease，the general clinic data and ocular findings were reviewed. Results： Of 74 patients， (31.1%)patients had accompanied by ocular manifestations ， and also 3 patients(4.1%) presented ocular syndrome with headache simultaneously the first symptoms of Moyamoya disease； Among the 74 patients， 24 patients(32.4%) had been disclosed unilateral or double stenosis of internal carotid artery by digital subtract angiography examination，only 3 patients had ocular syndrome。All positive eye findings included amaurosis fugax(13.3%)， hemianopia (17.4%)，decline or loss of the visual ability and lesions of visual fields 43.5%)， papillary size dysfunction (13.0%)， pupil direct/indirect light reflex(-)(13.0%)，ptosis(8.7%)，nystagmus (4.3%)， ocular dyscinesia (13.0%)， fossa orbitalis tenderness(8.7%)。CONCLUSIONS：As an ophthalmologist， should master the onset characteristics and clinical manifestation of Moyamoya disease. Early diagnosis and early treatment is very important for prevention the vision deterioration， especially to the patients with the ocular syndrome as the initial onset syndrome.

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