Acute disseminated encephalomyelitis (ADEM) is a monophasic demyelinating disorder of central nervous system occurring in children with a wide range of clinical manifestations after infection or vaccination. There are few case reports in literature, describing atypical presentations of ADEM with fever of unknown origin, autonomic dysfunction, complex movement disorders such as myoclonus, dystonia and chorea, acute psychosis and myocarditis. Here, we report four cases of ADEM with atypical features like uniocular blindness, myelin oligodendrocyte glycoprotein antibodies negative multiphasic disseminated encephalomyelitis, ADEM mimicking Guillain-Barre syndrome at presentation and isolated spinal ADEM. Treatment with high-dose steroids elicited an excellent neurological outcome in all patients. A high index of clinical suspicion along-with awareness of atypical features, magnetic resonance imaging and cerebrospinal fluid studies are of paramount importance in establishing ADEM diagnosis and initiation of early treatment for better outcome.

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儿童急性播散性脑脊髓炎的罕见和非典型表现：病例系列

急性播散性脑脊髓炎（ADEM）是儿童感染或接种疫苗后发生的具有广泛临床表现的中枢神经系统单相脱髓鞘疾病。文献中很少有病例报告描述了 ADEM 的非典型表现，包括不明原因的发热、自主神经功能障碍、复杂的运动障碍（如肌阵挛、肌张力障碍和舞蹈病）、急性精神病和心肌炎。在这里，我们报告了 4 例具有非典型特征的 ADEM 病例，如单眼失明、髓鞘少突胶质细胞糖蛋白抗体阴性多期播散性脑脊髓炎、就诊时模拟格林-巴利综合征的 ADEM 和孤立的脊髓 ADEM。大剂量类固醇治疗在所有患者中都产生了极好的神经学结果。