Mukhtiar Khairunnisa, Raza Mohammad, Ishaque Sidra, Maha Quratulain, Noor Ayesha
Khairunnisa Mukhtiar, Assistant Professor, Department of Pediatrics & Child Health, Section of Pediatric Neurology, Aga Khan University Hospital, Karachi, Pakistan.
Mohammad Raza, Instructor, Department of Pediatrics & Child Health, Section of Pediatric Neurology, Aga Khan University Hospital, Karachi, Pakistan.
Pak J Med Sci. 2024 Aug;40(7):1479-1484. doi: 10.12669/pjms.40.7.8015.
To determine the clinical spectrum, neuroimaging findings, and outcome of Acute Disseminated Encephalomyelitis (ADEM) in children.
We conducted a descriptive cross sectional study of all children aged 6 months to 18 years, diagnosed with ADEM at Aga Khan University Hospital, Karachi from January 2018 till December 2022.
This retrospective study enrolled 30 cases of ADEM, with a mean age of 6.43 ± 4.079, including 13 males and 17 females. The average hospital stay was 7.29 ± 4.379 days. The most common clinical features were fever, headache, and altered consciousness, while motor deficit was observed in 15 (53.5%) patients. Abnormal cerebrospinal fluid was found in 14 (46.6%) patients. Brain MRI identified bilateral and multifocal lesions in 22 (78.6%) patients, with brainstem lesions detected in 7 (25%) patients. Treatment included IV methylprednisolone (22; 73%), IVIG (9; 30%), or both (6; 20%). Clinical improvement was observed in 25 (89.3%) patients, with residual weakness present in eight (26%) patients at discharge. There was one reported death. Long-term complications included motor deficits, seizures, poor scholastic performance, and behavioral issues.
The clinical presentation of ADEM is variable, but the most common symptoms are fever, headache, and altered consciousness. Despite generally favorable outcome, long-term monitoring revealed that patients may experience motor deficits, seizures, cognitive impairment, and academic difficulties.
确定儿童急性播散性脑脊髓炎（ADEM）的临床谱、神经影像学表现及预后。
我们对2018年1月至2022年12月在卡拉奇阿迦汗大学医院诊断为ADEM的所有6个月至18岁儿童进行了描述性横断面研究。
这项回顾性研究纳入了30例ADEM病例，平均年龄为6.43±4.079岁，其中男性13例，女性17例。平均住院时间为7.29±4.379天。最常见的临床特征是发热、头痛和意识改变，15例（53.5%）患者出现运动功能障碍。14例（46.6%）患者脑脊液异常。脑部MRI显示22例（78.6%）患者有双侧和多灶性病变，7例（25%）患者检测到脑干病变。治疗包括静脉注射甲基强的松龙（22例；73%）、静脉注射免疫球蛋白（9例；30%）或两者联合使用（6例；20%）。25例（89.3%）患者临床症状改善，8例（26%）患者出院时仍有残留肌无力。有1例死亡报告。长期并发症包括运动功能障碍、癫痫发作、学业成绩差和行为问题。
ADEM的临床表现多样，但最常见的症状是发热、头痛和意识改变。尽管总体预后良好，但长期监测显示患者可能会出现运动功能障碍、癫痫发作、认知障碍和学业困难。
