Comert Serdar, Vitrinel Ayca, Gursu Hazim Alper, Deniz Neslihan Cicek, Akin Yasemin
Dr. Lutfi Kirdar Kartal Education and Research Hospital, Istanbul, Turkey.
Indian J Pediatr. 2006 Dec;73(12):1119-21. doi: 10.1007/BF02763061.
Subacute sclerosing panencephalitis (SSPE) is a chronic encephalitis of childhood and young adolescence due to persistent measles virus infection of the central nervous system. In majority of cases onset occurs from 5-15 years of age. In a nonimmunized population the average onset is 8 years. Children with SSPE had experienced natural infection with the rubeola virus at an early age, half before age 2 years. SSPE generally occurs 5-10 years after measles infection. In the early stages of the disease behavioral and personality changes is followed by myoclonic jerks and convulsions. In late stages dementia, stupor and coma develops. Diagnosis is achieved by typical clinical findings, measles antibody titer increase in cerebrospinal fluid (CSF) and serum, high amplitude, slow, sharp waves in EEG. Prognosis is poor and death ensues in about 3 yr after the diagnosis. Here it is presented a 7-years-old boy with involuntary movements in both hands, drop attacks while walking, ataxia and stupor. Due to suggestive radiological and clinical findings and a history of recent mumps infection he was thought to have acute disseminated encephalomyelitis initially and given treatment. But due to clinical deterioration and detection of anti measles IgG in serum and CSF, SSPE diagnosis was confirmed. With this SSPE case presenting initially as ADEM, the authors tried to emphasize that presentation of SSPE may clinically and radiologically be diverse and a thorough differential diagnosis is mandatory for a definite diagnosis.
亚急性硬化性全脑炎(SSPE)是一种发生于儿童和青少年时期的慢性脑炎,由麻疹病毒持续感染中枢神经系统所致。多数病例发病年龄在5至15岁之间。在未接种疫苗的人群中,平均发病年龄为8岁。患有SSPE的儿童在早年曾自然感染风疹病毒,半数在2岁之前。SSPE通常在麻疹感染后5至10年发生。在疾病早期,行为和性格改变之后会出现肌阵挛性抽搐和惊厥。在晚期会发展为痴呆、木僵和昏迷。通过典型的临床症状、脑脊液(CSF)和血清中麻疹抗体滴度升高以及脑电图中高波幅、慢波、锐波来进行诊断。预后较差,诊断后约3年死亡。本文介绍了一名7岁男孩,双手出现不自主运动、行走时跌倒发作、共济失调和木僵。由于影像学和临床症状提示以及近期有腮腺炎感染史,最初认为他患有急性播散性脑脊髓炎并给予了治疗。但由于临床病情恶化以及在血清和脑脊液中检测到抗麻疹IgG,确诊为SSPE。通过这个最初表现为ADEM的SSPE病例,作者试图强调SSPE在临床和影像学上的表现可能多种多样,为明确诊断必须进行全面的鉴别诊断。