PDF(Pubmed)
Abstract：
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: SSPE is associated with diverse movement disorders, predominantly hyperkinetic. The prevalence of dystonia suggests basal ganglia dysfunction.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: SSPE is associated with diverse movement disorders, predominantly hyperkinetic. The prevalence of dystonia suggests basal ganglia dysfunction.
摘要：
■亚急性硬化性全脑炎（SSPE）通常表现为周期性肌阵挛性；然而，一系列运动障碍，包括肌张力障碍，舞蹈病,震颤,和帕金森病也被描述过。这篇综述旨在评估SSPE中运动障碍的一系列，将它们与神经影像学检查结果相关联，疾病阶段，和患者结果。
■对已发表的病例报告和病例系列进行了全面审查，研究对象是表现出周期性肌阵挛性以外的运动障碍的SSPE患者。遵循PRISMA准则，并且该方案已在PROSPERO注册（2023CRD42023434650）。对多个数据库的全面搜索产生了37份报告，详细介绍了39例患者。Dyken的标准用于SSPE诊断，和国际运动障碍协会的定义被应用于运动障碍的分类。
■大多数患者是男性，平均年龄13.8岁。大约,80%的人缺乏可靠的疫苗接种史，39%曾感染过麻疹。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症。在64％的病例中发现疾病进展迅速，72％的疾病持续时间≤6个月。神经影像学显示T2/FLAIRMR高强度,主要是脑室周围,26％影响基底神经节/丘脑。脑活检显示炎症和神经退行性变化。超过一半的患者（56％）达到了运动静音状态或死亡。
■SSPE与多种运动障碍有关，主要是运动过度。肌张力障碍的患病率提示基底神经节功能障碍。
■对已发表的病例报告和病例系列进行了全面审查，研究对象是表现出周期性肌阵挛性以外的运动障碍的SSPE患者。遵循PRISMA准则，并且该方案已在PROSPERO注册（2023CRD42023434650）。对多个数据库的全面搜索产生了37份报告，详细介绍了39例患者。Dyken的标准用于SSPE诊断，和国际运动障碍协会的定义被应用于运动障碍的分类。
■大多数患者是男性，平均年龄13.8岁。大约,80%的人缺乏可靠的疫苗接种史，39%曾感染过麻疹。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症。在64％的病例中发现疾病进展迅速，72％的疾病持续时间≤6个月。神经影像学显示T2/FLAIRMR高强度,主要是脑室周围,26％影响基底神经节/丘脑。脑活检显示炎症和神经退行性变化。超过一半的患者（56％）达到了运动静音状态或死亡。
■SSPE与多种运动障碍有关，主要是运动过度。肌张力障碍的患病率提示基底神经节功能障碍。
