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同向性视野缺损作为克雅氏病的首发表现

Homonymous field defect as the first manifestation of Creutzfeldt-Jakob disease.

作者信息

Vargas M E, Kupersmith M J, Savino P J, Petito F, Frohman L P, Warren F A

机构信息

Department of Ophthalmology, New York University Medical Center, New York, USA.

出版信息

Am J Ophthalmol. 1995 Apr;119(4):497-504. doi: 10.1016/s0002-9394(14)71237-1.

DOI:10.1016/s0002-9394(14)71237-1
PMID:7709975
Abstract

PURPOSE

Although in the early stage of Creutzfeldt-Jakob disease most patients have obvious dementia, we found that the disease can be diagnosed in patients solely on the basis of a visual system disorder.

METHODS

We examined three patients who initially complained of a nonspecific, insidious visual disturbance.

RESULTS

The three patients were found to have Creutzfeldt-Jakob disease, confirmed by histopathologic analysis. Each patient had a homonymous hemianopsia on the first neuro-ophthalmologic examination. The initial neurologic, neuroimaging, and electrophysiologic examinations were not conclusive. The subsequent rapid deterioration in the neurologic status, including dementia and typical electroencephalographic changes, was suggestive of Creutzfeldt-Jakob disease.

CONCLUSION

At onset of Creutzfeldt-Jakob disease, patients may have a homonymous hemianopsia despite normal results of magnetic resonance imaging of the brain and neurologic examination.

摘要

目的

尽管在克雅氏病早期大多数患者有明显的痴呆症状,但我们发现仅根据视觉系统障碍就可对患者进行该病的诊断。

方法

我们检查了三名最初主诉有非特异性、隐匿性视觉障碍的患者。

结果

经组织病理学分析确诊这三名患者患有克雅氏病。在首次神经眼科检查时,每名患者均有同向偏盲。最初的神经学、神经影像学和电生理学检查结果并不明确。随后神经状态迅速恶化,包括出现痴呆和典型的脑电图变化,提示为克雅氏病。

结论

在克雅氏病发病时,尽管脑部磁共振成像和神经学检查结果正常,但患者可能会出现同向偏盲。

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