Mahadevan Anita, Vaidya Sunil R, Wairagkar Niteen S, Khedekar Deepika, Kovoor Jerry M E, Santosh Vani, Yasha T C, Satishchandra P, Ravi V, Shankar S K
Department of Neuropathology, National Institute of Mental Health and Neurosciences, Bangalore, Karnataka, India.
Neuropathology. 2008 Dec;28(6):621-6. doi: 10.1111/j.1440-1789.2008.00891.x. Epub 2008 Apr 1.
Subacute sclerosing panencephalitis (SSPE), a post-measles progressive neurological disorder is still common in India because of indifferent vaccination compliance. However, the acute fulminant form of SSPE is extremely rare. An unusual case of fulminant SSPE in an 18-year-old man from south India with an ultra-short course of 19 days presenting with hemiparesis in absence of myoclonus and progressive cognitive decline, is reported. MRI showed frontal and parieto-occipital demyelination extending to nuclear areas. Antimeasles antibodies were demonstrable in CSF and serum with oligoclonal bands in CSF despite normal CSF protein and cell count. At autopsy, unlike classical SSPE, oligodendroglia containing measles viral antigen was sparse despite florid necrotizing leukoencephalitis with acute demyelination. Measles virus was isolated from the brain with hypermutation in M gene confirming the diagnosis. Phylogenetic analysis of the viral genotype indicated that it belonged to D7 genotype which is considered rare in India.
亚急性硬化性全脑炎（SSPE）是一种麻疹后的进行性神经疾病，由于疫苗接种依从性差，在印度仍然很常见。然而，急性暴发型SSPE极为罕见。本文报道了一例来自印度南部的18岁男性患者，患有暴发型SSPE，病程极短，仅19天，表现为偏瘫，无肌阵挛，且伴有进行性认知衰退。MRI显示额叶和顶枕叶脱髓鞘，延伸至核区。脑脊液和血清中可检测到抗麻疹抗体，脑脊液中存在寡克隆带，尽管脑脊液蛋白和细胞计数正常。尸检时，与经典的SSPE不同，尽管存在 florid 坏死性白质脑炎伴急性脱髓鞘，但含有麻疹病毒抗原的少突胶质细胞稀少。从脑中分离出麻疹病毒，其M基因发生超突变，从而确诊。病毒基因型的系统发育分析表明，它属于D7基因型，在印度被认为是罕见的。
