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亚急性硬化性全脑炎的疾病修饰治疗:一个未知领域。

Disease-Modifying Therapy in Subacute Sclerosing Panencephalitis: An Area of Darkness.

作者信息

Garg Divyani, Sharma Suvasini

机构信息

Department of Neurology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India.

Department of Pediatrics (Neurology Division), Lady Hardinge Medical College, New Delhi, India.

出版信息

Ann Indian Acad Neurol. 2023 Jan-Feb;26(1):3-9. doi: 10.4103/aian.aian_655_22. Epub 2023 Jan 25.

Abstract

Subacute sclerosing panencephalitis (SSPE) is a neurodegenerative disorder because of the persistence of mutated measles virus in the central nervous system. Till date, no curative therapy has been established for SSPE. Multiple drugs have been tried to modify the disease process but have shown mild to moderate benefit at best. It is also challenging to attribute the relative success of some strategies described in single case reports because of the known phenomenon of spontaneous improvement in 5% of patients with SSPE. Critical gaps in understanding the pathophysiological processes involved exist. Current therapies such as interferon alfa require invasive strategies for administration by the intraventricular or intrathecal route, with varying dosage regimens. Oral therapies such as isoprinosine and ribavirin are expensive and not readily available in resource-constrained settings. Most of the evidence so far favors the use of combinational regimens. In this viewpoint, we critically summarize the current evidence on disease-modifying strategies in the context of our region.

摘要

亚急性硬化性全脑炎(SSPE)是一种神经退行性疾病,由突变的麻疹病毒在中枢神经系统持续存在所致。迄今为止,尚未确立针对SSPE的治愈性疗法。多种药物已被尝试用于改变疾病进程,但充其量仅显示出轻度至中度的益处。由于已知5%的SSPE患者会出现自发改善现象,因此在单一病例报告中描述的某些策略取得相对成功的原因也难以确定。在理解所涉及的病理生理过程方面存在关键差距。目前的疗法,如干扰素α,需要通过脑室内或鞘内途径进行侵入性给药,且给药方案各异。口服疗法,如异丙肌苷和利巴韦林,价格昂贵,在资源有限的环境中难以获得。迄今为止的大多数证据支持使用联合治疗方案。在本文观点中,我们在我们所在地区的背景下批判性地总结了当前关于疾病改善策略的证据。

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