Sudan Yeeshu Singh, Vinayan K P, Roy Arun Grace, Wagh Amarjeet, Kannoth Sudheeran, Patil Sandeep
Division of Pediatric Neurology, Department of Neurology, Amrita Institute of Medical Sciences, Amrita Viswa Vidyapeetham, Kochi, India.
Department of Neurology, Amrita Institute of Medical Sciences, Amrita Viswa Vidyapeetham, Kochi, India.
Indian J Pediatr. 2016 Nov;83(12-13):1367-1373. doi: 10.1007/s12098-016-2092-4. Epub 2016 Apr 18.
To describe the clinical characteristics of a cohort of south Indian children with probable autoimmune encephalopathy from a tertiary care academic hospital and to compare this data with the existing literature.
Patients with encephalopathy plus one or more of neuropsychiatric symptoms, seizures, movement disorder or cognitive dysfunction were identified. Common infectious causes were excluded. Clinical characteristics, investigations, management and outcome were analyzed.
Thirteen patients were included in the study; 12 were females (92.3 %) and mean age was 9.6 y. Most common presentation was behavior change (13 patients) followed by seizures (11 patients). Three patients showed lymphocytic pleocytosis in CSF and one patient had oligoclonal bands. Initial MRI was normal in all patients except in one. Most common EEG abnormality was mild background slowing. Only one child had ovarian tumor. S.NMDA receptor antibody was positive in 10 patients (83 %), and all of them received immunotherapy. Six out of 13 children were followed up for more than 1 y (mean - 21 mo). Recurrence was noted in 4 out of 6 patients (66 %). On last follow-up, good recovery was seen in 2 children (33 %), moderate disability in 3 (50 %) and severe disability in 1 (16 %).
The clinical characteristics and outcome of one of the largest single center cohort of Indian children with autoimmune encephalopathy is reported. Autoimmune encephalopathy should be considered as a differential diagnosis in the acute and subacute encephalopathies of childhood and treating pediatrician should be aware of this entity.
描述一家三级医疗学术医院中一组可能患有自身免疫性脑病的南印度儿童的临床特征，并将这些数据与现有文献进行比较。
确定患有脑病并伴有一种或多种神经精神症状、癫痫发作、运动障碍或认知功能障碍的患者。排除常见的感染病因。分析临床特征、检查、治疗及预后情况。
13例患者纳入研究；12例为女性（92.3%），平均年龄9.6岁。最常见的表现是行为改变（13例患者），其次是癫痫发作（11例患者）。3例患者脑脊液淋巴细胞增多，1例患者有寡克隆带。除1例患者外，所有患者的初始MRI均正常。最常见的脑电图异常是轻度背景慢波。仅1例儿童患有卵巢肿瘤。10例患者（83%）抗N-甲基-D-天冬氨酸受体（N.MDA）抗体呈阳性，所有这些患者均接受了免疫治疗。13例儿童中有6例随访超过1年（平均21个月）。6例患者中有4例（66%）复发。在最后一次随访时，2例儿童（33%）恢复良好，3例（50%）中度残疾，1例（16%）重度残疾。
报道了印度最大的单中心自身免疫性脑病儿童队列之一的临床特征和预后情况。自身免疫性脑病应被视为儿童急性和亚急性脑病的鉴别诊断之一，儿科医生应了解这一疾病实体。
