Subacute sclerosing panencephalitis (SSPE) is a lethal slow viral disease of the central nervous system caused by a defective measles virus. The onset is mostly in childhood, manifesting clinically as decline in academic performance, behavioural changes, motor dysfunction and myoclonus. Adult-onset SSPE is rare and can present as rapidly progressive dementia. We present a young man of Indian origin with adult-onset SSPE with rapidly progressive dementia but no localising neurological signs. The diagnostic clues were parieto-occipital white matter changes on MR brain scan and history of childhood fever with rash. High titres of antimeasles antibody in cerebrospinal fluid confirmed the diagnosis. The long latency from primary measles virus infection to symptom onset can be misleading in adults. SSPE should be considered in adults with dementia, especially in tropical countries where vaccination coverage is suboptimal.

中文翻译：

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成人发病的亚急性硬化性全脑炎


亚急性硬化性全脑炎（SSPE）是一种由有缺陷的麻疹病毒引起的中枢神经系统致命性慢病毒性疾病。发病多在儿童期，临床表现为学习成绩下降、行为改变、运动功能障碍和肌阵挛。成人发病的 SSPE 很少见，可表现为快速进展的痴呆。我们介绍了一名印度裔年轻人，患有成人发病的 SSPE，伴有快速进展的痴呆，但没有局部神经系统体征。诊断线索是磁共振脑部扫描的顶枕白质变化和儿童发烧伴皮疹史。脑脊液中抗麻疹抗体滴度高证实了诊断。从原发性麻疹病毒感染到症状出现的较长潜伏期可能会误导成年人。患有痴呆症的成年人应考虑 SSPE，特别是在疫苗接种覆盖率不理想的热带国家。