I型无脑回畸形的流行病学

Epidemiology of lissencephaly type I.

作者信息

de Rijk-van Andel J F, Arts W F, Hofman A, Staal A, Niermeijer M F

机构信息

Department of Neurology, Westeinde Hospital, The Hague, The Netherlands.

出版信息

Neuroepidemiology. 1991;10(4):200-4. doi: 10.1159/000110270.

DOI:10.1159/000110270

PMID:1745330

Abstract

In a cooperative study in The Netherlands 22 patients with lissencephaly type I were collected. In the period 1980-1988, the prevalence of lissencephaly type I in the Netherlands was 11.7 per million births (12.2 for females and 11.0 for males). The 5-year survival rate of patients with a complete or nearly complete agyria (grade 1 or 2) in this study was 54% compared to 91% in patients with a mixture of agyria with pachygyria or complete pachygyria (grade 3 or 4).

摘要

在荷兰的一项合作研究中，收集了22例Ⅰ型无脑回畸形患者。1980年至1988年期间，荷兰Ⅰ型无脑回畸形的患病率为每百万例出生11.7例（女性为12.2例，男性为11.0例）。本研究中无脑回完全或几乎完全缺失（1级或2级）患者的5年生存率为54%，相比之下，无脑回与巨脑回混合或完全巨脑回（3级或4级）患者的5年生存率为91%。