Government Medical College Trivandrum, Kerala, India.
Government Medical College Trivandrum, Kerala, India.
Pediatr Neurol. 2018 Dec;89:49-57. doi: 10.1016/j.pediatrneurol.2018.08.015. Epub 2018 Sep 21.
We studied the long-term outcome of Acute disseminated encephalomyelitis (ADEM).
We performed a retrospective cohort study among children diagnosed with ADEM (fulfilling IPMSSG criteria). Major outcome variables were motor deficit, scholastic underperformance, and behavioral abnormality.
The inclusion criteria were fulfilled by 102 children. Three died in hospital. The follow-up ranged from one to 10 years (median 4 years). Motor deficit was seen in 17(17.2%), attention deficit in 25 (25.3%), behavioral abnormality in 13(13.1%), persistent seizures in seven (7%) invididuals and poor learning skills in 22 (22.2%). Recurrence of demyelination occurred in seven (7.1%). Two individuals had a recurrent demyelinating disorder (a chronic relapsing demyelinating disorder) that could not be classified as multiple sclerosis (MS), two had ADEM with sequential optic neuritis and three had multiphasic ADEM. At follow-up, the mean (SD) modified Rankin Scale (mRS) score was 0.556 (1.36) and Expanded Disability Status Scale score was 1.71(2.22). On multivariate analysis, the mRS score at discharge (p<0.01) and thalamic lesions on magnetic resonance imaging (MRI) (p<0.01) were associated with motor sequelae; poor learning skills with ADEM with concomitant polyneuropathy (p<0.02); and behavioral abnormality with tumefactive demyelination (p<0.02).
Children who had ADEM may have motor or cognitive sequelae, seizures or recurrent demyelinating events on follow-up. We identified a few risk factors for these sequelae. Factors that affected outcome on discharge from hospital did not affect chances of having long-term sequelae. On follow-up, none of the children fulfilled the diagnostic criteria for MS, suggesting that the chance of conversion of ADEM to MS is less likely.
我们研究了急性播散性脑脊髓炎（ADEM）的长期预后。
我们对符合 IPMSSG 标准的 ADEM 患儿进行了回顾性队列研究。主要结局变量为运动障碍、学业成绩不良和行为异常。
102 名患儿符合纳入标准，其中 3 例患儿在住院期间死亡。随访时间为 1 至 10 年（中位数 4 年）。17 例（17.2%）患儿出现运动障碍，25 例（25.3%）患儿出现注意力缺陷，13 例（13.1%）患儿出现行为异常，7 例（7.1%）患儿出现持续性癫痫发作，22 例（22.2%）患儿学习技能差。7 例（7.1%）患儿发生脱髓鞘复发。2 例患儿出现复发性脱髓鞘疾病（慢性复发性脱髓鞘疾病），不能归类为多发性硬化症（MS），2 例患儿 ADEM 伴序贯视神经炎，3 例患儿多相 ADEM。随访时，平均（SD）改良 Rankin 量表（mRS）评分 0.556（1.36），扩展残疾状况量表（EDSS）评分 1.71（2.22）。多变量分析显示，出院时 mRS 评分（p<0.01）和 MRI 上的丘脑病变（p<0.01）与运动后遗症相关；伴有多发性神经病的 ADEM 与学习能力差相关（p<0.02）；肿瘤样脱髓鞘与行为异常相关（p<0.02）。
ADEM 患儿在随访中可能有运动或认知后遗症、癫痫发作或复发性脱髓鞘事件。我们确定了一些这些后遗症的危险因素。影响出院时预后的因素并不影响长期后遗症的发生几率。随访中，无一例患儿符合 MS 的诊断标准，提示 ADEM 向 MS 转化的可能性较小。
