Jagtap Sujit Abajirao, Nair M D, Kambale Harsha J
Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India.
Ann Indian Acad Neurol. 2013 Oct;16(4):631-3. doi: 10.4103/0972-2327.120497.
Subacute sclerosing panencephalitis (SSPE) is a rare chronic, progressive encephalitis affecting primarily children and young adults, caused by a persistent infection of immune resistant measles virus. The aim of the present study is to describe the clinical profile and natural history of patients with SSPE.
We collected data of patients with SSPE during 2004-2010 who fulfilled Dyken's criteria. We analyzed demographical, clinical, electrophysiological, and imaging features.
Study included 34 patients, 26 (76.5%) males with age of onset from 3 to 31 years. Twenty one patients were below 15 years of age formed childhood SSPE and 13 above 15 years of age constituted adult onset group. 85.3% had low-socioeconomic status. Eleven received measles vaccination and seven were unvaccinated. 59.9% patients had measles history. Most common presenting symptom was scholastic backwardness (52.5%) followed by seizures (23.5%). Three patients each had cortical blindness, macular degeneration, decreased visual acuity, and optic atrophy. Electroencephalographic (EEG) showed long interval periodic complexes and cerebrospinal fluid anti-measles antibody was positive in all. Magnetic resonance imaging was done in 70.5% with was abnormal in 52.5%. Mean incubation period of SSPE after measles was 9.6 years. The follow-up duration was 1-10 years, (average of 2 years). Only one patient died from available data of follow-up, 9 were stable and 10 deteriorated in the form of progression of staging.
SSPE is common in low-socioeconomic status. The profile of adult onset did not differ from childhood onset SSPE, except for a longer interval between measles infection and presence of the ophthalmic symptom as presenting feature in adult onset group.
亚急性硬化性全脑炎（SSPE）是一种罕见的慢性进行性脑炎，主要影响儿童和青年，由免疫抗性麻疹病毒的持续感染引起。本研究的目的是描述SSPE患者的临床特征和自然病史。
我们收集了2004年至2010年期间符合戴肯标准的SSPE患者的数据。我们分析了人口统计学、临床、电生理和影像学特征。
研究纳入34例患者，26例（76.5%）为男性，发病年龄为3至31岁。21例年龄在15岁以下的患者构成儿童SSPE组，13例年龄在15岁以上的患者构成成人发病组。85.3%的患者社会经济地位较低。11例接受过麻疹疫苗接种，7例未接种。59.9%的患者有麻疹病史。最常见的首发症状是学业落后（52.5%），其次是癫痫发作（23.5%）。3例患者分别出现皮质盲、黄斑变性、视力下降和视神经萎缩。脑电图（EEG）显示长间隔周期性复合波，脑脊液抗麻疹抗体均为阳性。70.5%的患者进行了磁共振成像，其中52.5%异常。麻疹后SSPE的平均潜伏期为9.6年。随访时间为1至10年（平均2年）。从现有随访数据来看，仅1例患者死亡，9例病情稳定，10例病情分期进展恶化。
SSPE在社会经济地位较低的人群中较为常见。成人发病型SSPE的特征与儿童发病型无差异，只是成人发病组麻疹感染与出现眼部症状作为首发特征之间的间隔时间更长。
