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[伴有神经症状的罕见葡萄膜炎形式:伏格特-小柳-原田葡萄膜脑膜脑炎综合征]

[Rare form of uveitis with neurological symptoms: the Vogt-Koyanagi-Harada uveomeningoencephalitic syndrome].

作者信息

Kätterer C, Kaeser H E, Steiger U

机构信息

Neurologische Universitätsklinik, Kantonsspital Basel.

出版信息

Schweiz Med Wochenschr. 1992 Feb 22;122(8):269-74.

PMID:1542780
Abstract

A 51-year-old Italian woman developed migraine-like headaches with increasing frequency 5 years after menopause. The simultaneous onset of severe bilateral uveitis, neurological signs such as vertigo, hearing loss, small sensomotor hemisyndrome, cognitive brain dysfunction and lymphocyte liquor pleocytosis confirmed the diagnosis of Vogt-Koyanagi-Harada syndrome, a rare disease of probably autoimmune origin with destruction of pigment containing neuroepithelium. Other vasculitic diseases, especially those of infectious or rheumatologic origin, were excluded. Systemic corticoid treatment suppressed the activity of the illness but on dose reduction relapses occurred. Additional immunosuppressive medication prevented uveitic relapses and made it possible to reduce the corticoid dose.

摘要

一名51岁的意大利女性在绝经后5年出现偏头痛样头痛,且发作频率不断增加。同时出现的严重双侧葡萄膜炎、眩晕、听力丧失、轻度感觉运动性偏身综合征、认知性脑功能障碍等神经学症状以及淋巴细胞性脑脊液增多症,确诊为Vogt-小柳-原田综合征,这是一种可能起源于自身免疫、罕见的疾病,会破坏含色素的神经上皮。排除了其他血管炎疾病,尤其是那些由感染或风湿引起的疾病。全身性皮质类固醇治疗抑制了疾病的活动,但在减量时复发。额外的免疫抑制药物预防了葡萄膜炎复发,并使得减少皮质类固醇剂量成为可能。

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