Cowan L D, Hudson L S
Department of Biostatistics and Epidemiology, College of Public Health, University of Oklahoma Health Sciences Center, Oklahoma City.
J Child Neurol. 1991 Oct;6(4):355-64. doi: 10.1177/088307389100600412.
Few population-based studies of infantile spasms have been done, and most reports have not included comparison groups. In spite of these limitations, this review attempts to summarize what is known about the distribution, etiology, and natural history of infantile spasms in populations; discusses the limitations of current data; and includes suggestions for further population-based research. Most estimates of the incidence of infantile spasms are between 0.25 and 0.42 per 1000 live births per year. Among children less than 10 years of age, the annual prevalence of infantile spasms is 0.14 to 0.19 per 1000. The peak age at onset of spasms is 4 to 6 months, and there appears to be a slight excess of male cases. The etiology of infantile spasms is unknown for 40% to 50% of affected children. Selected syndromes (eg, Aicardi syndrome, Down syndrome, etc) and inherited disorders (eg, tuberous sclerosis) may account for as many as one third of cases. Other factors, such as intrauterine infection, "birth injury," and head trauma have not been systematically evaluated, and thus, their contribution to the etiology of infantile spasms is uncertain. Areas for future etiologic research include controlled studies of immunologic factors and in utero and postnatal infections and further exploration of the interrelationship between infantile spasms and Lennox-Gastaut syndrome.
基于人群的婴儿痉挛症研究较少,且大多数报告未纳入对照组。尽管存在这些局限性,本综述仍试图总结关于婴儿痉挛症在人群中的分布、病因及自然史的已知情况;讨论当前数据的局限性;并提出进一步开展基于人群研究的建议。大多数对婴儿痉挛症发病率的估计为每年每1000例活产中有0.25至0.42例。在10岁以下儿童中,婴儿痉挛症的年患病率为每1000人中有0.14至0.19例。痉挛发作的高峰年龄为4至6个月,男性病例似乎略多。40%至50%的患婴病因不明。特定综合征(如Aicardi综合征、唐氏综合征等)和遗传性疾病(如结节性硬化症)可能占病例的三分之一。其他因素,如宫内感染、“产伤”和头部外伤尚未得到系统评估,因此它们对婴儿痉挛症病因的贡献尚不确定。未来病因学研究的领域包括对免疫因素以及宫内和产后感染的对照研究,以及进一步探索婴儿痉挛症与Lennox-Gastaut综合征之间的相互关系。