Department of Medicine, University of Toronto, 190 Elizabeth Street R. Fraser Elliott Wing, Toronto, M5G 2C4, Canada.
Child Health Evaluative Sciences Research Program, SickKids Research Institute, The Hospital for Sick Children, Toronto, Canada.
Orphanet J Rare Dis. 2018 Nov 29;13(1):216. doi: 10.1186/s13023-018-0952-x.
Infantile spasms represent the catastrophic, age-specific seizure type associated with acute and long-term neurological morbidity. However, due to rarity and heterogenous determination, there is persistent uncertainty of its pathophysiological and epidemiological characteristics. The purpose of the current study was to address a historically suspected latitudinal basis of infantile spasms incidence, and to interrogate a geographical basis of epidemiology, including the roles of latitude and other environmental factors, using meta-analytic and -regression methods.
A systematic search was performed in Ovid MEDLINE and Embase for primary reports on infantile spasms incidence and prevalence epidemiology.
One thousand fifteen studies were screened to yield 54 eligible publications, from which 39 incidence figures and 18 prevalence figures were extracted. The pooled incidence was 0.249 cases/1000 live births. The pooled prevalence was 0.015 cases/1000 population. Univariate meta-regression determined a continental effect, with Europe demonstrating the highest onset compared from Asia (OR = 0.51, p = 0.004) and from North America (OR = 0.50, p = 0.004). Latitude was also positively correlated with incidence globally (OR = 1.02, p < 0.001). Sub-analyses determined a particularly elevated Scandinavian incidence compared to the rest of world (OR = 1.88, p < 0.001), and lack of latitudinal effect with Scandinavian exclusion (p = 0.10). Metrics of healthcare quality did not predict incidence. Multiple meta-regression determined that latitude was the key predictor of incidence (OR = 1.02, p = 0.001).
This is the first systematic epidemiological study of infantile spasms. Limitations included lack of Southern hemispheric representation, insufficient study selection and size to support some sub-continental analyses, and lack of accessible ethnic and healthcare quality data. Meta-analyses determined a novel, true geographical difference in incidence which is consistent with a latitudinal and/or ethnic contribution to epileptogenesis. These findings justify the establishment of a global registry of infantile spasms epidemiology to promote future systematic studies, clarify risk factors, and expand understanding of the pathophysiology.
婴儿痉挛症代表与急性和长期神经发育障碍相关的特定于年龄的灾难性癫痫发作类型。然而，由于其罕见性和异质性，其病理生理学和流行病学特征仍存在持续的不确定性。本研究的目的是解决婴儿痉挛症发病率的历史上可疑的纬度基础，并使用荟萃分析和回归方法探讨地理流行病学基础，包括纬度和其他环境因素的作用。
在 Ovid MEDLINE 和 Embase 中进行了系统搜索，以查找婴儿痉挛症发病率和流行率流行病学的原始报告。
筛选了 1105 项研究，得出了 54 项合格的出版物，从中提取了 39 项发病率数据和 18 项患病率数据。汇总的发病率为 0.249 例/1000 例活产儿。汇总的患病率为 0.015 例/1000 人。单变量荟萃回归确定了大陆效应，欧洲的发病高峰高于亚洲（OR=0.51，p=0.004）和北美（OR=0.50，p=0.004）。纬度与全球发病率呈正相关（OR=1.02，p<0.001）。亚分析确定了斯堪的纳维亚地区的发病率明显高于世界其他地区（OR=1.88，p<0.001），并且排除斯堪的纳维亚地区后没有纬度效应（p=0.10）。医疗保健质量指标不能预测发病率。多元回归确定纬度是发病率的关键预测因素（OR=1.02，p=0.001）。
这是第一项关于婴儿痉挛症的系统流行病学研究。其局限性包括南半球代表性不足、选择和大小不足以支持某些次大陆分析、以及缺乏可获取的种族和医疗保健质量数据。荟萃分析确定了一种新颖的、真正的发病率地理差异，这与癫痫发生的纬度和/或种族因素一致。这些发现证明了建立婴儿痉挛症流行病学全球登记处的合理性，以促进未来的系统研究、阐明危险因素，并扩大对病理生理学的认识。
