Progressive multifocal leukoencephalopathy (PML) associated with HIV Clade C--is not uncommon.
Progressive multifocal leukoencephalopathy (PML) associated with HIV Clade C--is not uncommon.
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与 HIV C 型相关的进行性多灶性白质脑病 (PML) 并不罕见。
DOI：
10.1007/s13365-013-0168-8
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发表时间：
2013
影响因子：
3.2
通讯作者：
Shankar,SK
中科院分区：
文献类型：
--
作者：
Netravathi,M;Mahadevan,Anita;Satishchandra,Parthasarathy;Shobha,N;Mailankody,Pooja;Kandavel,Thennarasu;Jitender,Saini;Anantaram,G;Nagarathna,S;Govekar,S;Ravikumar,BV;Ravi,V;Shankar,SK
进行性多灶性白质脑病(PML)是由JC病毒引起的一种罕见的亚急性中枢神经系统脱髓鞘疾病。对艾滋病毒C分支流行国家的PML的研究很少。我们试图研究印度HIV C分支患者的PML的临床、神经影像和病理特征。这是一项前瞻性和回顾性研究，于2001年1月至2012年5月在印度的一家三级护理神经科转诊中心进行。诊断为“确定”(经组织病理学或脑脊液JCV-PCR证实)或“可能”(经MRI脑成像证实)。2001年1月至2012年5月，55名PML患者被诊断为PML。38名患者获得了完整的数据[平均年龄39 ± 8.9a；病程82.1 ± 74.7d]。PML在我们研究所发现的2.8%的HIV队列中流行。偏瘫是最常见的症状(44.7%)，其次是共济失调(36.8%)。其中20例可明确诊断。18例仍“可能”，MRI显示多灶性对称性病变，T1低信号，T2/FLAIR高信号。立体定向活检(n= 11)可见脱髓鞘、增大的少突胶质细胞核内包涵体和星形胶质细胞增多。免疫组织化学显示JC病毒抗原存在于少突胶质细胞核和星形细胞胞浆中。与HIV分支B相关的PML在临床、放射学或病理特征上没有明显差异，只有一半的患者考虑到临床怀疑PML。因此，高度怀疑指数对诊断至关重要。印度和西方国家的PML的临床、放射学和病理特征无显著差异。
Progressive multifocal leukoencephalopathy (PML) is a rare, subacute, demyelinating disease of the central nervous system caused by JC virus. Studies of PML from HIV Clade C prevalent countries are scarce. We sought to study the clinical, neuroimaging, and pathological features of PML in HIV Clade C patients from India. This is a prospective cum retrospective study, conducted in a tertiary care Neurological referral center in India from Jan 2001 to May 2012. Diagnosis was considered “definite” (confirmed by histopathology or JCV PCR in CSF) or “probable” (confirmed by MRI brain). Fifty-five patients of PML were diagnosed between January 2001 and May 2012. Complete data was available in 38 patients [mean age 39 ± 8.9 years; duration of illness—82.1 ± 74.7 days). PML was prevalent in 2.8 % of the HIV cohort seen in our Institute. Hemiparesis was the commonest symptom (44.7 %), followed by ataxia (36.8 %). Definitive diagnosis was possible in 20 cases. Eighteen remained “probable” wherein MRI revealed multifocal, symmetric lesions, hypointense on T1, and hyperintense on T2/FLAIR. Stereotactic biopsy (n= 11) revealed demyelination, enlarged oligodendrocytes with intranuclear inclusions and astrocytosis. Immunohistochemistry revelaed the presence of JC viral antigen within oligodendroglial nuclei and astrocytic cytoplasm. No differences in clinical, radiological, or pathological features were evident from PML associated with HIV Clade B. Clinical suspicion of PML was entertained in only half of the patients. Hence, a high index of suspicion is essential for diagnosis. There are no significant differences between clinical, radiological, and pathological picture of PML between Indian and Western countries.