Kalra V, Passi G R
Department of Pediatrics, All India Institute of Medical Sciences, New Delhi.
Brain Dev. 1998 Jan;20(1):14-7. doi: 10.1016/s0387-7604(97)00080-6.
To analyse the clinical characteristics of patients with childhood epileptic encephalopathies, a retrospective study was carried out on paediatric neurology clinic records of a tertiary hospital. Forty-five children with childhood epileptic encephalopathies were identified. Patients were classified according to the international classification of epilepsies and epileptic syndromes, data were collected regarding age at onset, perinatal problems, presence of psychomotor retardation, radiological findings, etiology and response to therapy. Characteristics of responders versus non-responders were compared. The majority had West syndrome (29/45 or 64.4%). Of the total, 37/45 or 82.2% were symptomatic. The etiologic factors identified included perinatal problems in 24/45 or 53.3%, one patient with tuberous sclerosis and one with Aicardi's syndrome. Psychomotor retardation was seen in 95.5%. Cranial CT scan was normal in 11/26 or 42.3%. Abnormalities included infarcts (4/26), generalised atrophy and hydrocephalus (3/26), porencephalic cysts (2/26) and agenesis of corpus callosum, tuberous sclerosis, gliosis and subdural effusion (one each). Mean follow-up was 18 months and 71.4% responded to ACTH. There was no significant difference between responders and non-responders.
为分析儿童癫痫性脑病患者的临床特征，对一家三级医院儿科神经科门诊记录进行了一项回顾性研究。共识别出45例儿童癫痫性脑病患者。根据国际癫痫及癫痫综合征分类对患者进行分类，收集有关发病年龄、围产期问题、精神运动发育迟缓情况、影像学检查结果、病因及治疗反应的数据。比较了有反应者与无反应者的特征。大多数患者患有韦斯特综合征（29/45，占64.4%）。其中，37/45（82.2%）为症状性癫痫。确定的病因包括围产期问题（24/45，占53.3%）、1例结节性硬化症患者和1例艾卡迪综合征患者。95.5%的患者存在精神运动发育迟缓。26例患者中11例（42.3%）头颅CT扫描正常。异常情况包括梗死（4/26）、广泛性萎缩和脑积水（3/26）、脑穿通畸形囊肿（2/26）以及胼胝体发育不全、结节性硬化症、胶质增生和硬膜下积液（各1例）。平均随访18个月，71.4%的患者对促肾上腺皮质激素有反应。有反应者与无反应者之间无显著差异。
