Laboratory for Clinical Research in Neuroinfection, National Institute of Infectious Diseases Evandro Chagas, FIOCRUZ, Rio de Janeiro, Brazil; Neurology Service, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil.
Laboratory of Viral Pathogenesis, National Institute of Infectious Diseases Evandro Chagas, FIOCRUZ, Rio de Janeiro, Brazil.
J Neurol Sci. 2017 Oct 15;381:321-324. doi: 10.1016/j.jns.2017.09.002. Epub 2017 Sep 4.
Progressive multifocal leukoencephalopathy (PML) is lytic infection of oligodendrocytes caused by JC virus (JCV). While PML incidence in developing countries has decreased after the introduction of combination antiretroviral therapy (cART), data in developing countries is scarce and limited to few cohorts. We described the epidemiological and clinical profile of a group of Brazilian HIV infected patients with PML in the cART era. A total of 27 patients were included in the study. The median age at PML onset was 42years (range: 27-67years) and 18 (66.7%) were men. The median CD4+ T cell count at the time of diagnosis was 67cells/mm and the median HIV viral load was 27,000copies/ml. Motor deficits were the most common early manifestations (44%). Seizures occurred in 37% of the patients and 9 (33.3%) had PML associated with immune reconstitution inflammatory syndrome (IRIS). Mortality was 33% and lower age at PML onset was associated with survival (p: 0.013). Our results are in accordance with previous published series of PML cases. Factors such as genetic background, regional JCV subtype differences, death from other diseases and underdiagnosis may explain the low prevalence of reported PML cases in developing countries.
进行性多灶性白质脑病(PML)是由 JC 病毒(JCV)引起的少突胶质细胞溶解性感染。虽然在发展中国家引入联合抗逆转录病毒疗法(cART)后,PML 的发病率有所下降,但发展中国家的数据仍然稀缺,且仅限于少数队列。我们描述了 cART 时代巴西一组 HIV 感染患者的 PML 流行病学和临床特征。共有 27 名患者纳入研究。PML 发病时的中位年龄为 42 岁(范围:27-67 岁),18 名(66.7%)为男性。诊断时的中位 CD4+T 细胞计数为 67 个/毫米,中位 HIV 病毒载量为 27,000 拷贝/ml。运动障碍是最常见的早期表现(44%)。癫痫发作发生在 37%的患者中,9 例(33.3%)与免疫重建炎症综合征(IRIS)相关的 PML。死亡率为 33%,发病年龄越小,存活率越高(p:0.013)。我们的结果与之前发表的 PML 病例系列相符。遗传背景、区域 JCV 亚型差异、死于其他疾病和漏诊等因素可能解释了发展中国家报告的 PML 病例低流行率。