Department of Neurology, King George's Medical University, Lucknow, India.
Department of Microbiology, King George's Medical University, Lucknow, India.
Tremor Other Hyperkinet Mov (N Y). 2024 May 14;14:27. doi: 10.5334/tohm.875. eCollection 2024.
Subacute Sclerosing Panencephalitis (SSPE) typically presents with periodic myoclonus; however, a spectrum of movement disorders including dystonia, chorea, tremor, and parkinsonism have also been described. This review aims to evaluate the array of movement disorders in SSPE, correlating them with neuroimaging findings, disease stages, and patient outcomes.
A comprehensive review of published case reports and case series was conducted on patients with SSPE exhibiting movement disorders other than periodic myoclonus. PRISMA guidelines were followed, and the protocol was registered with PROSPERO (2023 CRD42023434650). A comprehensive search of multiple databases yielded 37 reports detailing 39 patients. Dyken's criteria were used for SSPE diagnosis, and the International Movement Disorders Society definitions were applied to categorize movement disorders.
The majority of patients were male, with an average age of 13.8 years. Approximately, 80% lacked a reliable vaccination history, and 39% had prior measles infections. Dystonia was the most common movement disorder (49%), followed by parkinsonism and choreoathetosis. Rapid disease progression was noted in 64% of cases, with a disease duration of ≤6 months in 72%. Neuroimaging showed T2/FLAIR MR hyperintensities, primarily periventricular, with 26% affecting the basal ganglia/thalamus. Brain biopsies revealed inflammatory and neurodegenerative changes. Over half of the patients (56%) reached an akinetic mute state or died.
SSPE is associated with diverse movement disorders, predominantly hyperkinetic. The prevalence of dystonia suggests basal ganglia dysfunction.
亚急性硬化性全脑炎（SSPE）通常表现为周期性肌阵挛；然而，也已经描述了一系列运动障碍，包括肌张力障碍、舞蹈症、震颤和帕金森病。本综述旨在评估 SSPE 中的运动障碍谱，将其与神经影像学发现、疾病阶段和患者预后相关联。
对表现为周期性肌阵挛以外的运动障碍的 SSPE 患者的已发表病例报告和病例系列进行了全面的文献回顾。遵循 PRISMA 指南，并在 PROSPERO（2023 CRD42023434650）上注册了方案。对多个数据库进行了全面搜索，共获得了 37 份详细描述 39 名患者的报告。使用 Dyken 标准诊断 SSPE，并应用国际运动障碍学会的定义对运动障碍进行分类。
大多数患者为男性，平均年龄为 13.8 岁。大约 80%的患者没有可靠的疫苗接种史，39%的患者有既往麻疹感染史。肌张力障碍是最常见的运动障碍（49%），其次是帕金森病和舞蹈手足徐动症。64%的病例病情进展迅速，72%的病例病程≤6 个月。神经影像学显示 T2/FLAIR MR 高信号，主要为脑室周围，26%的病例影响基底节/丘脑。脑活检显示炎症和神经退行性改变。超过一半的患者（56%）进入无动性缄默状态或死亡。
SSPE 与多种运动障碍相关，主要为多动性。肌张力障碍的患病率提示基底节功能障碍。
