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进行性多灶性白质脑病作为结节病未经治疗时的一种罕见致命并发症。

Progressive multi-focal leukoencephalopathy as a rare lethal complication in untreated sarcoidosis.

作者信息

Hohlfeld Sabine K, Günthard Huldrych F, Zeitz Jonas, Locher Pascal, Bachli Esther

机构信息

Department of Internal Medicine, University Hospital Zurich, Zurich, Switzerland.

出版信息

BMJ Case Rep. 2012 Jun 28;2012:bcr0320114036. doi: 10.1136/bcr.03.2011.4036.

Abstract

Progressive multi-focal leukoencephalopathy (PML) is a demyelinating disease of the central nervous system caused by reactivation of John Cunningham virus. It is an opportunistic infection affecting patients who are severely immunocompromised due to an underlying disease or secondary to immunosuppressive therapy. To date, no effective antiviral therapy has been established, though several substances are being investigated. The authors present the case of a 39-year-old previously healthy patient who was diagnosed with PML and sarcoidosis stage III without having received prior immunosuppressive treatment. The patient did not respond to treatment with mirtazapine, which has been used empirically, and deceased shortly after diagnosis.

摘要

进行性多灶性白质脑病(PML)是一种由约翰·坎宁安病毒再激活引起的中枢神经系统脱髓鞘疾病。它是一种机会性感染,影响因基础疾病或继发于免疫抑制治疗而严重免疫功能低下的患者。迄今为止,尚未确立有效的抗病毒治疗方法,不过有几种药物正在研究中。作者报告了一例39岁的既往健康患者,该患者在未接受过免疫抑制治疗的情况下被诊断为PML和III期结节病。该患者对经验性使用的米氮平治疗无反应,诊断后不久死亡。

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