Feng Y
Peking Union Medical College Hospital, Beijing.
Zhonghua Shen Jing Jing Shen Ke Za Zhi. 1992 Feb;25(1):25-7, 61-2.
This paper is a summary of our observations on 105 cases of infantile spasms. The age of onset was around six months after birth, but the patients came for treatment mainly about one year after onset. Fever of unknown cause, asphyxia, birth injury, infection of the central nervous system, tuberous sclerosis, phenylketonuria and recent immunization etc. were possible etiology. Clinically, it is characterized by head nodding, mental retardation, myoclonic jerks and various neurologic deficits. EEG findings showed classical or modified arrhythmia or other epileptiform patterns. About one third of 22 cases examined had abnormal brain stem auditory evoked potentials. Among 42 patients who underwent CT scanning before ACTH treatment, 18 were normal and 7 abnormal; during ACTH treatment 3 normal and 4 abnormal; after completion of treatment, 4 normal and 6 abnormal, suggesting no further atrophy of the brain. Examination of trace elements of the hair by proton-induced X-ray emission (PIXE) method in 23 patients revealed a significant difference in lead, calcium and zinc contents between patients and 101 controls, but no statistical difference in iron and copper contents between the two groups. Sodium valproate, prednisone and ACTH appear to be effective in the treatment of infantile spasms. Eight patients fully recovered, and they can go to school without difficulty. Many patients derived various degrees of improvement of the satisfaction of their parents. Two patients were still amended and often attacked by myoclonus. The effects, side effects of these drugs, and the possible pathogenesis were discussed.
本文是对我们观察的105例婴儿痉挛症病例的总结。发病年龄约在出生后6个月左右,但患者主要在发病后约1年前来就诊。不明原因发热、窒息、产伤、中枢神经系统感染、结节性硬化症、苯丙酮尿症及近期免疫接种等均可能为病因。临床上,其特征为点头、智力发育迟缓、肌阵挛抽搐及各种神经功能缺损。脑电图表现为典型或变异型节律紊乱或其他癫痫样波型。22例接受检查的患者中约三分之一脑干听觉诱发电位异常。42例在促肾上腺皮质激素(ACTH)治疗前行CT扫描的患者中,18例正常,7例异常;ACTH治疗期间3例正常,4例异常;治疗结束后,4例正常,6例异常,提示脑无进一步萎缩。用质子激发X射线发射(PIXE)法检测23例患者头发微量元素,发现患者与101例对照者铅、钙和锌含量有显著差异,但两组铁和铜含量无统计学差异。丙戊酸钠、泼尼松和ACTH似乎对婴儿痉挛症治疗有效。8例患者完全康复,能顺利上学。许多患者有不同程度改善,其父母满意度较高。2例患者仍有发作,常出现肌阵挛。讨论了这些药物的疗效、副作用及可能的发病机制。