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首页> 外文期刊>Journal of neurology >Drug-associated progressive multifocal leukoencephalopathy: a clinical, radiological, and cerebrospinal fluid analysis of 326 cases
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Drug-associated progressive multifocal leukoencephalopathy: a clinical, radiological, and cerebrospinal fluid analysis of 326 cases

机译：药物相关的进行性多灶性白质脑病：326例临床，影像学和脑脊液分析

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The implementation of a variety of immunosuppressive therapies has made drug-associated progressive multifocal leukoencephalopathy (PML) an increasingly prevalent clinical entity. The purpose of this study was to investigate its diagnostic characteristics and to determine whether differences herein exist between the multiple sclerosis (MS), neoplasm, post-transplantation, and autoimmune disease subgroups. Reports of possible, probable, and definite PML according to the current diagnostic criteria were obtained by a systematic search of PubMed and the Dutch pharmacovigilance database. Demographic, epidemiologic, clinical, radiological, cerebrospinal fluid (CSF), and histopathological features were extracted from each report and differences were compared between the disease categories. In the 326 identified reports, PML onset occurred on average 29.5 months after drug introduction, varying from 14.2 to 37.8 months in the neoplasm and MS subgroups, respectively. The most common overall symptoms were motor weakness (48.6 %), cognitive deficits (43.2 %), dysarthria (26.3 %), and ataxia (24.1 %). The former two also constituted the most prevalent manifestations in each subgroup. Lesions were more often localized supratentorially (87.7 %) than infratentorially (27.4 %), especially in the frontal (64.1 %) and parietal lobes (46.6 %), and revealed enhancement in 27.6 % of cases, particularly in the MS (42.9 %) subgroup. Positive JC virus results in the first CSF sample were obtained in 63.5 %, while conversion after one or more negative outcomes occurred in 13.7 % of cases. 52.2 % of patients died, ranging from 12.0 to 83.3 % in the MS and neoplasm subgroups, respectively. In conclusion, despite the heterogeneous nature of the underlying diseases, motor weakness and cognitive changes were the two most common manifestations of drug-associated PML in all subgroups. The frontal and parietal lobes invariably constituted the predilection sites of drug-related PML lesions.
机译：各种免疫抑制疗法的实施已使药物相关的进行性多灶性白质脑病（PML）成为越来越普遍的临床实体。这项研究的目的是调查其诊断特征，并确定多发性硬化症（MS），肿瘤，移植后和自身免疫性疾病亚组之间是否存在差异。通过对PubMed和Dutch药物警戒数据库进行系统搜索，可获得根据当前诊断标准可能，可能和确定的PML的报告。从每份报告中提取人口统计学，流行病学，临床，放射学，脑脊液（CSF）和组织病理学特征，并比较疾病类别之间的差异。在326份已确定的报告中，PML发作平均在药物引入后29.5个月发生，在肿瘤和MS亚组中分别从14.2到37.8个月不等。最常见的总体症状是运动无力（48.6％），认知缺陷（43.2％），构音障碍（26.3％）和共济失调（24.1％）。前两个也构成每个亚组中最普遍的表现。病变多见于幕上（87.7％），而不是fra下（27.4％），尤其是额叶（64.1％）和顶叶（46.6％），并且在27.6％的病例中有所增强，特别是在MS（42.9％）亚组。在首个CSF样本中获得JC病毒阳性结果的率为63.5％，而在发生一个或多个阴性结果后的转化率为13.7％。 52.2％的患者死亡，MS和肿瘤亚组分别为12.0％至83.3％。总之，尽管潜在疾病具有异质性，但运动无力和认知变化是所有亚组中与药物相关的PML的两种最常见表现。额叶和顶叶始终构成药物相关性PML病变的好发部位。

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