Dale Russell C
Neurosciences Unit, Institute of Child Health and Great Ormond Street Hospital NHS trust, and the Department of Neuroinflammation, Institute of Neurology, London, United Kingdom.
Semin Pediatr Infect Dis. 2003 Apr;14(2):90-5. doi: 10.1053/spid.2003.127225.
Acute disseminated encephalomyelitis (ADEM) is a monophasic inflammatory disorder of the central nervous system (CNS). Unlike viral encephalitis, microorganisms do not invade the CNS. Instead, ADEM is a postinfectious disease mediated by auto-reactive cells or molecules. Clinical characteristics of ADEM are consistent with disseminated involvement of the CNS, including encephalopathy and pyramidal, cerebellar, and brainstem signs. Bilateral optic neuritis and transverse myelitis are particularly suggestive of demyelinating diseases such as ADEM. Unlike viral encephalitis, seizures rarely are a prominent symptom. The most useful diagnostic investigation is magnetic resonance neuroimaging that commonly shows multifocal lesions throughout the brain and spinal cord. As ADEM is an immune-mediated disorder, treatment includes immunomodulatory therapies (particularly steroids), although no clinical trials have been performed to define the most efficacious agent. In view of the treatment differences between ADEM and viral encephalitis, being familiar with ADEM is essential for pediatricians managing acute neurological disorders.
急性播散性脑脊髓炎(ADEM)是一种中枢神经系统(CNS)的单相性炎症性疾病。与病毒性脑炎不同,微生物不会侵袭中枢神经系统。相反,ADEM是一种由自身反应性细胞或分子介导的感染后疾病。ADEM的临床特征与中枢神经系统的播散性受累一致,包括脑病以及锥体束、小脑和脑干体征。双侧视神经炎和横贯性脊髓炎尤其提示为ADEM等脱髓鞘疾病。与病毒性脑炎不同,癫痫很少是突出症状。最有用的诊断检查是磁共振神经成像,其通常显示大脑和脊髓中多处病灶。由于ADEM是一种免疫介导的疾病,治疗包括免疫调节疗法(尤其是类固醇),尽管尚未进行临床试验来确定最有效的药物。鉴于ADEM和病毒性脑炎在治疗上的差异,熟悉ADEM对于处理急性神经系统疾病的儿科医生至关重要。