Independent distribution between tauopathy secondary to subacute sclerotic panencephalitis and measles virus: An immunohistochemical analysis in autopsy cases including cases treated with aggressive antiviral therapies.
Independent distribution between tauopathy secondary to subacute sclerotic panencephalitis and measles virus: An immunohistochemical analysis in autopsy cases including cases treated with aggressive antiviral therapies.
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DOI：
10.1111/bpa.13069
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发表时间：
2022-11
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亚急性脊髓全脑炎（SSPE）是暴露于麻疹病毒后的一种难治性神经系统疾病。最近，SSPE病例已经用抗病毒治疗进行了治疗，但疗效数据尚不确定。在SSPE病例的脑组织中已经报道了异常的tau积累，但是很少有报道对此进行了充分的讨论。本研究包括5例尸检确诊为SSPE的病例。受试者年龄或疾病持续时间范围分别为7.6 - 40.9岁或0.5 - 20.8年。例3和例4已接受抗病毒治疗。所有评价病例均显示明显脑萎缩伴脑室扩张;此外，在大脑白色物质中观察到明显脱髓鞘伴神经胶质增生。脑干、小脑和脊髓相对保存。在未治疗病例的脑干被盖、新皮质和/或边缘皮质中观察到抗麻疹病毒的免疫反应性（IR），但在两种治疗病例中很少观察到。从大脑到脊髓广泛观察到活化的小胶质细胞，并且在病例之间没有显著差异。以3-和4-重复tau组合为特征的神经元缠结主要在眼神经核、蓝斑和边缘皮质中观察到。针对磷酸化tau的IR主要见于扣带回、眼神经核和脑桥被盖，并且往往在病程较长的病例中频繁观察到，但也往往随着神经元损失而沿着减少，如病例5，其病程最长。由于磷酸化tau蛋白的分布与麻疹病毒的分布无关，因此推断SSPE后的tau蛋白病是麻疹引发的弥漫性脑炎症的结果，而不是麻疹病毒的直接结果。此外，抗病毒治疗似乎抑制麻疹病毒，但不能抑制tau蛋白病的进展。
Subacute sclerotic panencephalitis (SSPE) is a refractory neurological disorder after exposure to measles virus. Recently, SSPE cases have been treated with antiviral therapies, but data on the efficacy are inconclusive. Abnormal tau accumulation has been reported in the brain tissue of SSPE cases, but there are few reports in which this is amply discussed. Five autopsied cases diagnosed as definite SSPE were included in this study. The subject age or disease duration ranged from 7.6 to 40.9 years old or from 0.5 to 20.8 years, respectively. Cases 3 and 4 had been treated with antiviral therapies. All evaluated cases showed marked brain atrophy with cerebral ventricle dilatation; additionally, marked demyelination with fibrillary gliosis were observed in the cerebral white matter. The brainstem, cerebellum, and spinal cord were relatively preserved. Immunoreactivity (IR) against measles virus was seen in the brainstem tegmentum, neocortex, and/or limbic cortex of the untreated cases but was rarely seen in the two treated cases. Activated microglia were broadly observed from the cerebrum to the spinal cord and had no meaningful difference among cases. Neurofibrillary tangles characterized by a combination of 3‐ and 4‐repeat tau were observed mainly in the oculomotor nuclei, locus coeruleus, and limbic cortex. IR against phosphorylated tau was seen mainly in the cingulate gyrus, oculomotor nuclei, and pontine tegmentum, and tended to be observed frequently in cases with long disease durations but also tended to decrease along with neuronal loss, as in Case 5, which had the longest disease duration. Since the distribution of phosphorylated tau was independent from that of measles virus, the tauopathy following SSPE was inferred to be the result of diffuse brain inflammation triggered by measles rather than a direct result of measles virus. Moreover, antiviral therapies seemed to suppress measles virus but not the progression of tauopathy.
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影响因子：
15.1
作者：
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通讯作者：
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作者：
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11.2
作者：
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