Subacute sclerosing panencephalitis presenting as acute disseminated encephalomyelitis
Subacute sclerosing panencephalitis presenting as acute disseminated encephalomyelitis
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DOI：
10.1007/bf02763061
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发表时间：
2006-12-01
影响因子：
4.3
通讯作者：
Akin, Yasemin
中科院分区：
文献类型：
--
作者：
Comert, Serdar;Vitrinel, Ayca;Akin, Yasemin
亚急性硬化性全脑炎（SSPE）是儿童和青少年的慢性脑炎，是由中枢神经系统持续的麻疹病毒感染引起的。在大多数病例中，发病年龄为5-15岁。(1)在未接种疫苗的人群中，平均发病时间为8年。患有SSPE的儿童在很小的时候就经历过风疹病毒的自然感染，其中一半在2岁之前。(2) SSPE一般发生在麻疹感染后5 ~ 10年。在疾病的早期阶段，行为和性格的改变伴随着肌阵挛性抽搐和抽搐。在老年痴呆症的晚期，会出现麻木和昏迷。临床表现典型，脑脊液和血清麻疹抗体滴度增高，脑电图高振幅、慢速、尖波。预后差，诊断后约3年死亡。这是一个7岁的男孩，双手不自主运动，走路时摔倒，共济失调和昏迷。由于可疑的放射学和临床表现以及最近的腮腺炎感染史，最初认为他患有急性播散性脑脊髓炎并给予治疗。但由于临床恶化及血清和脑脊液中检测到抗麻疹IgG，证实了SSPE的诊断。由于这个SSPE病例最初表现为ADEM，作者试图强调SSPE的临床和影像学表现可能是多种多样的，彻底的鉴别诊断是明确诊断的必要条件。
Subacute sclerosing panencephalitis (SSPE) is a chronic encephalitis of childhood and young adolescence due to persistant measles virus infection of the central nervous system. In majority of cases onset occurs from 5-15 years of age.(1) 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.(2) 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.