Garg Meenal, Arora Anshita, Kulkarni Shilpa D, Hegde Anaita Udwadia, Shah Krishnakumar N
Department of Pediatric Neurology, Surya Hospitals, Jaipur, Rajasthan, India.
Division of Pediatric Neurology, Department of Pediatric Neurosciences, Bai Jerbai Wadia Hospital for Children, Mumbai, Maharashtra, India.
J Neurosci Rural Pract. 2022 Feb 10;13(2):315-320. doi: 10.1055/s-0041-1740612. eCollection 2022 Apr.
Subacute sclerosing panencephalitis (SSPE) is a devastating neurodegenerative disease occurring as a complication of measles infection that is still prevalent in low-resource countries. Clinical and electrographical variability in SSPE can lead to diagnostic delays. Children diagnosed with SSPE in a tertiary care pediatric hospital in India in a period of 8 years were included in the study. The diagnosis was established on the basis of Dyken's criteria. The demographic data, clinical presentations, investigations, treatment approaches, and outcomes were reviewed and recorded. Thirty-four patients were included in the analysis. Average age at symptom onset was 7 years, 5 months. Majority of the children were not vaccinated for measles. Most patients (80%) presented with stage 2 of illness. Nearly 25% presented with atypical clinical features. Myoclonus was the most predominant feature seen after diagnosis. Electroencephalography (EEG) was the most useful investigation for suspecting the diagnosis. All patients showed deterioration in neurological status with time and 20% died during follow-up. Atypical presentations of SSPE must be recognized in areas with high incidence to institute timely treatment and establish prognosis. EEG findings were found to be the most important indicator for diagnosis. Measles eradication will pave the way for elimination of this dreaded disease.
亚急性硬化性全脑炎(SSPE)是一种毁灭性的神经退行性疾病,是麻疹感染的并发症,在资源匮乏国家仍很常见。SSPE的临床和脑电图表现具有变异性,可能导致诊断延迟。
本研究纳入了印度一家三级儿科医院在8年期间诊断为SSPE的儿童。诊断依据戴肯标准确定。对人口统计学数据、临床表现、检查、治疗方法和结果进行了回顾和记录。
34例患者纳入分析。症状出现时的平均年龄为7岁5个月。大多数儿童未接种麻疹疫苗。大多数患者(80%)处于疾病2期。近25%的患者表现为非典型临床特征。肌阵挛是诊断后最主要的特征。脑电图(EEG)是怀疑诊断时最有用的检查。所有患者的神经功能状态均随时间恶化,20%在随访期间死亡。
在发病率高的地区必须认识到SSPE的非典型表现,以便及时治疗并确定预后。EEG结果被发现是诊断的最重要指标。消除麻疹将为消除这种可怕疾病铺平道路。