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患儿女,52天。于1993年10月22日以癫痫而入院。30天前患儿在医院婴儿室出院后即发现每次睡前先双眼上翻,发出一声尖叫,继而抽搐,强直性痉挛性发作,持续约5分钟后入睡,每天2~3次。患儿系第1胎1产,足月剖腹产,产后正常,人工喂养。母孕期正常。入院查体:神经系统表现为轻度角弓反张状,双下肢轻微剪刀步,四肢肌张力略强。脑电图检查呈典型暴发抑制波。诊断为大田原综合征。临床治疗10天无变化而要求转院,后到北京儿童医院仍确诊为大田原综合征,随访患儿于1994年2月12日死亡。讨论:大田原综合征是一种恶性癫痫,1976年首先由日本学者大田原提出。本病又称早期婴儿型癫痫性脑病,是年龄依赖性癫痫性脑病中最具代表性的一
Children with children, 52 days. In October 22, 1993 to epilepsy and admission. 30 days before discharge in the hospital baby room after the discovery of the children each time before going to bed double-fold, uttered a scream, and then convulsions, tonic spastic attack, continued to sleep about 5 minutes later, 2 or 3 times a day. Children born in the first 1 births, full-term caesarean section, normal postpartum, artificial feeding. Mother during pregnancy normal. Admission examination: nervous system showed a slight angle arch anti-Zhang, a pair of lower limbs slightly scissor steps, limb muscle tension slightly stronger. EEG examination showed typical outbreak suppression wave. Diagnosis of Otahara syndrome. Clinical treatment of 10 days no change in the request transfer, after the Beijing Children’s Hospital is still diagnosed with Ota original syndrome, children were followed up on February 12, 1994 death. Discussion: Otahara syndrome is a malignant epilepsy, first proposed in 1976 by the Japanese scholar OTA original. The disease, also known as early infant epileptic encephalopathy, is the most representative of age-dependent epileptic encephalopathy