Michałowicz R, Ignatowicz R, Kmieć T, Kuczyński D
Zespołu neurologii Dzieciecej Pomnika, Szpitala Centrum Zdrowia Dziecka, Warszawie.
Pol Tyg Lek. 1994;49(4-5):93-5.
West syndrome is a from of epileptic attacks of infants. Diagnosis of West syndrome includes: presence of the sudden violent flexion of the trunk and limbs, psychomotoric development retardation, especially after the onset of attacks, abnormal EEG records, and therapeutical problems. Clinical course and results of therapy were analysed in 66 children with West syndrome (39 boys and 27 girls). Children were divided into four groups, depending on etiology of the disease. Group 1 included 39 children with lesions to CNS during pregnancy; group 2-8 children with developmental CNS disorders, group 3-6 children with a history of encephalitis or meningitis, and group 4-13 children in whom etiology of West syndrome was unclear. Patients were treated with Synacthen-Depot in a daily dose of 0.03 mg/kg combined with other anti-epileptic agents. The most difficult to treat were those children in whom West syndrome occurred below 6 months of life, were psychomotor retarded before the onset of symptoms, hormonal treatment was introduced with delay, there were additional seizures of different etiology, and there were frequently recurrent infections.
韦斯特综合征是婴儿癫痫发作的一种形式。韦斯特综合征的诊断包括：躯干和四肢突然剧烈屈曲、精神运动发育迟缓（尤其是在发作后）、脑电图记录异常以及治疗问题。对66例韦斯特综合征患儿（39例男孩和27例女孩）的临床病程和治疗结果进行了分析。根据疾病病因，将患儿分为四组。第一组包括39例孕期中枢神经系统受损的患儿；第二组8例患有中枢神经系统发育障碍的患儿，第三组6例有脑炎或脑膜炎病史的患儿，第四组13例韦斯特综合征病因不明的患儿。患者接受每日剂量为0.03 mg/kg的长效促肾上腺皮质激素治疗，并联合其他抗癫痫药物。最难治疗的是那些在6个月以下发病、症状出现前有精神运动发育迟缓、激素治疗延迟、有不同病因的额外发作且频繁反复感染的患儿。
