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一名62岁免疫功能正常女性的进行性多灶性白质脑病。

Progressive multifocal leukoencephalopathy in a 62-year-old immunocompetent woman.

作者信息

Gourineni Venkata C, Juvet Tristan, Kumar Yogesh, Bordea Doru, Sena Kanaga N

机构信息

Department of Internal Medicine, Bridgeport Hospital, Yale New Haven Health, 267 Grant Street, Bridgeport, CT 06610, USA.

Department of Radiology, Bridgeport Hospital, Yale New Haven Health, 267 Grant Street, Bridgeport, CT 06610, USA.

出版信息

Case Rep Neurol Med. 2014;2014:549271. doi: 10.1155/2014/549271. Epub 2014 Feb 23.

Abstract

Progressive multifocal encephalopathy (PML) is a rare demyelinating disease that typically presents in immunodeficient patients. We report a case of a previously healthy 62-Year-Old woman who suffered from an unsteady gait, throbbing headaches, and progressive left-sided weakness and numbness. Stroke was initially suspected based on imaging and symptoms. A series of follow-up magnetic resonance images of the brain showed a right parietal lesion growing in size as the patient became unable to walk and experienced increasing lethargy and confusion. A biopsy of the lesion was positive for the John Cunningham virus (JCV). A diagnosis of PML was made and she was started on mefloquine. No improvement was seen on this treatment and her condition worsened. Although PML remains uncommon in immunocompetent individuals, it cannot be ruled out based on their immune status. Although the exact cause remains uncertain, underlying or transient states of immunosuppression may be responsible for reactivation of the JCV in these patients.

摘要

进行性多灶性白质脑病(PML)是一种罕见的脱髓鞘疾病,通常发生于免疫缺陷患者。我们报告一例病例,患者为一名62岁既往健康女性,出现步态不稳、搏动性头痛以及进行性左侧肢体无力和麻木。基于影像学检查和症状,最初怀疑为中风。一系列脑部磁共振成像随访显示,随着患者无法行走且嗜睡和意识模糊加重,右侧顶叶病变体积增大。病变活检显示约翰·坎宁安病毒(JCV)阳性。诊断为PML后,开始给予甲氟喹治疗。该治疗未见改善,患者病情恶化。尽管PML在免疫功能正常个体中仍不常见,但不能基于其免疫状态排除该病。尽管确切病因仍不确定,但潜在或短暂的免疫抑制状态可能是这些患者中JCV重新激活的原因。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0dfb/3965952/f49d70570190/CRINM2014-549271.001.jpg

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