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脑眼型惠普尔病:从死亡边缘到确诊的62年漫长历程

Cerebral ocular Whipple's disease: a 62-year odyssey from death to diagnosis.

作者信息

Knox D L, Green W R, Troncoso J C, Yardley J H, Hsu J, Zee D S

机构信息

Wilmer Ophthalmological Institute, Johns Hopkins Medical Institutions, Baltimore, MD, USA.

出版信息

Neurology. 1995 Apr;45(4):617-25. doi: 10.1212/wnl.45.4.617.

Abstract

A 47-year-old white man with dementia, supranuclear ophthalmoplegia, and myoclonic ocular and facial jerks died in 1931. The case report in 1936 by Ford and Walsh diagnosed encephalitis. In 1993, we made a clinical diagnosis of Whipple's disease on the basis of the 1936 publication. We restudied the pathologic material and found, in addition to extensive encephalitis, PAS-positive material in only the eye, brain, spinal cord, and pituitary. Electron microscopy demonstrated free and intracytoplasmic microorganisms in the eye and brain. We review the history of cerebral ocular Whipple's disease and the implications from this case, which occurred before the development of antibiotics.

摘要

一名患有痴呆、核上性眼肌麻痹以及眼部和面部肌阵挛的47岁白人男性于1931年去世。1936年福特和沃尔什的病例报告诊断为脑炎。1993年,我们根据1936年的出版物对该病例做出了惠普尔病的临床诊断。我们重新研究了病理材料,发现除广泛的脑炎外,仅在眼、脑、脊髓和垂体中存在PAS阳性物质。电子显微镜检查在眼和脑中发现了游离的和胞质内的微生物。我们回顾了脑眼型惠普尔病的病史以及该病例(发生在抗生素出现之前)所带来的启示。

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