Chatterjee Anirban, Datta Supratim
Department of Pediatric Medicine, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India.
J Pediatr Neurosci. 2016 Jul-Sep;11(3):241-243. doi: 10.4103/1817-1745.193352.
Acute disseminated encephalomyelitis (ADEM) is an idiopathic inflammatory demyelinating disease of the central nervous system (CNS) and considered mostly a monophasic course. Recurrence of ADEM is rare entity, posing diagnostic dilemma with multiple sclerosis (MS). There were no definite diagnostic criteria or established treatment for ADEM. International Pediatric MS Study Group laid down first consensus definition. We report a boy presented with recurrent episodes of fever, paraparesis, seizure, and unconsciousness. Magnetic resonance imaging (MRI) brain revealed the recurrence of lesions in the same brain site of the previous event. The first and second events were at age of three and five, respectively, with no neurological deficit, clinically and MRI, in between period of 24 months. We found that he responded dramatically both episodes with intravenous immunoglobulin treatment and no CNS deficit was found on 3-year prospective evaluation to exclude MS. We diagnosed recurrent ADEM.
急性播散性脑脊髓炎（ADEM）是一种中枢神经系统（CNS）的特发性炎性脱髓鞘疾病，大多被认为是单相病程。ADEM复发是罕见情况，这给多发性硬化症（MS）带来了诊断难题。目前尚无明确的ADEM诊断标准或既定治疗方法。国际儿童MS研究小组制定了首个共识定义。我们报告一名男孩，出现发热、双下肢轻瘫、癫痫发作和意识丧失的反复发作。脑部磁共振成像（MRI）显示在前次事件相同脑区出现病变复发。首次和第二次事件分别发生在3岁和5岁时，在24个月期间临床及MRI检查均无神经功能缺损。我们发现他在两次发作时接受静脉注射免疫球蛋白治疗均有显著反应，且在3年的前瞻性评估中未发现中枢神经系统缺损以排除MS。我们诊断为复发性ADEM。
