麻疹亚急性硬化性全脑炎(SSPE):一种仍然存在且仍然神秘的致命疾病。病史、诊断和假设]。

Medecine sciences : M/S Pub Date : 2022-06-01 Epub Date: 2022-06-29 DOI:10.1051/medsci/2022081
Pierre Lebon, Antoinette Gelot, Shen-Ying Zhang, Jean-Laurent Casanova, Jean-Jacques Hauw
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引用次数: 1

摘要

亚急性硬化性全脑炎是麻疹的晚期并发症,由于疫苗接种不足,在麻疹流行期间仍然存在。在回顾历史后,讨论了SSEP的诊断标准和病理生理学的重要性。大量关于先天免疫和干扰素应答参数的研究倾向于显示细胞免疫活性降低。根据不同形式的疾病的出版物,提出了几种假设:先天性,围产期,与急性包涵性脑炎(AIE)相似的短潜伏期形式,快速发展形式,免疫功能低下形式,甚至成人形式。已经确定了家族形式,这表明是遗传原因。根据潜伏期的持续时间,对两组患者进行了个体化治疗,并对这些患者的外显子组进行了回顾性和前瞻性分析。对相关基因的了解将有助于了解SSPE和其他晚期神经RNA病毒感染的病理生理学。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Measles subacute sclerosing panencephalitis (SSPE): A still present and still mysterious fatal disease. History, Diagnosis and Assumptions].

Subacute sclerosing panencephalitis, a late complication of measles, is still present during epidemics of this disease due to insufficient vaccination. After a historical review, the importance of the diagnostic criteria and the pathophysiology of SSEP are discussed. Numerous studies on the parameters of innate immunity and interferon responses tend to show a decrease in the activity of cellular immunity. Several hypotheses are formulated based on the publications of the different forms of the disease: Congenital, perinatal, forms with short incubation similar to acute inclusion encephalitis (AIE), rapidly evolving forms, forms of the immunocompromised, or even adults. Familial forms have been identified, suggesting a genetic cause. Based on the duration of the latency period, two groups have been individualized, prompting a retrospective and prospective analysis of the exomes of these patients. The knowledge of the genes involved should be useful for the understanding of the pathophysiology of SSPE and other late neurological RNA virus infections.

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