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[涉及中枢神经系统白质的伊尔斯病]

[Eales' disease involving central nervous system white matter].

作者信息

Antigüedad A, Zarranz J J

机构信息

Servicio y Cátedra de Neurología, Hospital de Cruces, Universidad del País Vasco, Vizcaya.

出版信息

Neurologia. 1994 Aug-Sep;9(7):307-10.

PMID:7946429
Abstract

Eales' disease (ED) is a rare condition characterized by repeated retinal and vitreous hemorrhages. The only extraocular involvement described occasionally in the literature is neurological. Histologically, vasculitis in ED is usually restricted to the eye, but occasionally involves the central nervous system, where demyelinizing lesions may also occur. We present a 34-year-old male with ED and subclinical central nervous system involvement. Craneal magnetic resonance images (MR) suggested demyelinization; brainstem auditory and somatosensory evoked potentials were abnormal. There was moderate pleocytosis in CSF and intratecal production of immunoglobulins with oligoclonal bands. Follow-up over a period of 2.5 years showed no clinical, MR or CSF changes in spite of continued opthamological impairment. Little is known about factors that affect the development or not of demyelinizing lesions in ED patients with neurological involvement demonstrated by intratecal production of immunoglobulins. Identification of such factors may contribute to our understanding of other diseases, such as multiple sclerosis.

摘要

伊尔斯氏病(ED)是一种罕见疾病,其特征为视网膜和玻璃体反复出血。文献中偶尔描述的唯一眼外受累情况是神经方面的。组织学上,ED中的血管炎通常局限于眼部,但偶尔会累及中枢神经系统,在中枢神经系统也可能出现脱髓鞘病变。我们报告一名34岁男性患有ED且有亚临床中枢神经系统受累。头颅磁共振成像(MR)提示脱髓鞘;脑干听觉和体感诱发电位异常。脑脊液中有中度淋巴细胞增多,鞘内产生免疫球蛋白并伴有寡克隆带。尽管存在持续的眼科损害,但经过2.5年的随访,未发现临床、MR或脑脊液有变化。对于鞘内产生免疫球蛋白证明有神经受累的ED患者中影响脱髓鞘病变发生与否的因素知之甚少。识别这些因素可能有助于我们对其他疾病(如多发性硬化症)的理解。

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