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进行性多灶性白质脑病:慢性B细胞淋巴细胞白血病的一种并发症

[Progressive multifocal leukoencephalopathy: a complication in chronic B-cell lymphatic leukaemia].

作者信息

van Kernebeek Caroline R, Ünal Esra, Schaar Cees G, Bienfait H P

机构信息

Gelre ziekenhuizen, Apeldoorn. Afd. Neurologie.

Contact: Caroline R. van Kernebeek (

出版信息

Ned Tijdschr Geneeskd. 2020 Mar 12;164:D4182.

PMID:32267644
Abstract

BACKGROUND

Progressive multifocal leukoencephalopathy (PML) is a rare infectious cause of sub-acute neurological symptoms, and occurs predominantly in immunocompromised patients. PML is caused by reactivation of the JC virus.

CASE DESCRIPTION

A 79-year-old man with a history of chronic B-cell lymphatic leukaemia (B-CLL) presented at our hospital with a neurological deficit of the left side of his body. He was initially diagnosed with a right-hemisphere stroke. Two months later he returned with progressive paresis and on an MRI of the brain we saw an increase in abnormalities of the white matter. On suspicion of PML we conducted PCR for JC virus on cerebrospinal fluid (CSF), which was negative. Histopathological investigations of a brain biopsy confirmed the diagnosis of PML, four months after he first presented.

CONCLUSION

PML is a rare cause of sub-acute neurological symptoms. PML can be difficult to diagnose as a PCR of CSF for JC virus in the early stages of PML can give a false negative result. If PML is suspected, histological investigation of a brain biopsy is necessary.

摘要

背景

进行性多灶性白质脑病(PML)是亚急性神经症状的一种罕见感染病因,主要发生于免疫功能低下的患者。PML由JC病毒再激活引起。

病例描述

一名79岁男性,有慢性B细胞淋巴细胞白血病(B-CLL)病史,因身体左侧神经功能缺损入住我院。他最初被诊断为右半球中风。两个月后,他因进行性麻痹再次就诊,脑部MRI显示白质异常增多。怀疑为PML后,我们对其脑脊液(CSF)进行了JC病毒PCR检测,结果为阴性。首次就诊四个月后,脑活检的组织病理学检查确诊为PML。

结论

PML是亚急性神经症状的罕见病因。PML可能难以诊断,因为在PML早期对CSF进行JC病毒PCR检测可能会得出假阴性结果。如果怀疑为PML,进行脑活检的组织学检查是必要的。

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