Nair Sruthi S, Vysakha K V, Menon Ramshekhar N, Sundaram Soumya
Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, India.
Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, India
Pract Neurol. 2021 Apr 13. doi: 10.1136/practneurol-2020-002880.
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.
亚急性硬化性全脑炎（SSPE）是一种由缺陷型麻疹病毒引起的致命性中枢神经系统慢病毒疾病。发病多在儿童期，临床症状表现为学业成绩下降、行为改变、运动功能障碍和肌阵挛。成人起病的SSPE较为罕见，可表现为快速进展性痴呆。我们报告了一名来自印度的年轻男性，患有成人起病的SSPE并伴有快速进展性痴呆，但无定位性神经系统体征。诊断线索为脑部磁共振扫描显示顶枕叶白质改变以及儿童期发热伴皮疹病史。脑脊液中高滴度的抗麻疹抗体确诊了该病。从原发性麻疹病毒感染到症状出现的长潜伏期在成人中可能会产生误导。对于患有痴呆的成人，尤其是在疫苗接种覆盖率欠佳的热带国家，应考虑到SSPE的可能。
