Gowda Vykuntaraju K, Shetty Deepthi, Madivala Bhaskar V, Benakappa Naveen, Benakappa Asha
Department of Pediatric Neurology, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India.
Department of Pediatrics, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India.
J Pediatr Neurosci. 2019 Apr-Jun;14(2):76-81. doi: 10.4103/jpn.JPN_62_18.
Demyelinating disorders of central nervous system are rare childhood disorders that cause significant physical and cognitive disabilities. Early diagnosis and appropriate treatment determines prognosis and outcome.
The objective of this work was to study clinical profile, investigative and radiological features, treatment, and outcome of children with demyelinating disorders.
A retrospective chart review of 32 children with demyelinating disorders admitted in a tertiary-care pediatric hospital from Bangalore between November 2013 and October 2017 was conducted. Sociodemographic data, clinical features, cerebrospinal fluid (CSF) findings, radiological features, treatment received, and outcome were collected and results were analyzed.
Among 32 patients of demyelinating disorders, majority were acute disseminated encephalomyelitis (ADEM) (15, 46.9%), followed by transverse myelitis (9, 28.1%), multiple sclerosis (MS) (3, 9.4%), optic neuritis (3, 9.4%), and neuromyelitis optica (NMO) (2, 6.2%). Mean age of presentation was 7.8 years. Among 32 patients, 15 were male (47%). In patients with ADEM, the mean age of presentation was 5.5 years. Two patients had tumefactive ADEM and 10 recovered after steroid therapy. In patients with MS, the mean age of presentation was 10.6 years; one patient died and the condition of two patients improved. In patients with transverse myelitis, the mean age of presentation was 8.9 years and seven patients recovered. In a patient with NMO, CSF was positive for antibody to aquaporin-4. The child improved with steroids and intravenous immunoglobulin. Three patients with optic neuritis were treated with steroids and they recovered.
Even though demyelinating disorders are rare in pediatric age group, one should be aware of this entity as early diagnosis and treatment improves outcome. Most common among them is ADEM.
中枢神经系统脱髓鞘疾病是罕见的儿童疾病，会导致严重的身体和认知残疾。早期诊断和适当治疗决定预后和结局。
本研究旨在探讨儿童脱髓鞘疾病的临床特征、检查及影像学表现、治疗方法和结局。
对2013年11月至2017年10月期间在班加罗尔一家三级儿科医院收治的32例脱髓鞘疾病患儿进行回顾性病历分析。收集社会人口统计学数据、临床特征、脑脊液（CSF）检查结果、影像学表现、接受的治疗及结局，并对结果进行分析。
在32例脱髓鞘疾病患者中，多数为急性播散性脑脊髓炎（ADEM）（15例，46.9%），其次是横贯性脊髓炎（9例，28.1%）、多发性硬化（MS）（3例，9.4%）、视神经炎（3例，9.4%）和视神经脊髓炎（NMO）（2例，6.2%）。平均发病年龄为7.8岁。32例患者中，15例为男性（47%）。ADEM患者的平均发病年龄为5.5岁。2例患者为肿胀型ADEM，10例患者经类固醇治疗后康复。MS患者的平均发病年龄为10.6岁；1例患者死亡，2例患者病情改善。横贯性脊髓炎患者的平均发病年龄为8.9岁，7例患者康复。1例NMO患者的脑脊液水通道蛋白4抗体呈阳性。该患儿经类固醇和静脉注射免疫球蛋白治疗后病情改善。3例视神经炎患者接受类固醇治疗后康复。
尽管脱髓鞘疾病在儿童年龄组中较为罕见，但由于早期诊断和治疗可改善结局，因此应提高对此类疾病的认识。其中最常见的是ADEM。
