多小脑回
小头畸形
智力残疾
胼胝体
癫痫
外显子组测序
发育不良
表型
发育不全
医学
皮质发育不良
生物
神经科学
遗传学
解剖
基因
作者
N Laflamme,Valérie Triassi,Laurence Martineau,Dènahin Hinnoutondji Toffa,Laurent Létourneau‐Guillon,Annie Laplante,Patrick Cossette,Éric Samarut,Martine Tétreault,Dang Khoa Nguyen
摘要
We studied three brothers and a maternal half-brother featuring global developmental delay, mild to moderate intellectual disability, epilepsy, microcephaly, and strabismus. All had bilateral perisylvian and perirolandic polymicrogyria, while some also had malformations of the hippocampus (malrotation and dysplasia), cerebellum (heterotopias and asymmetric aplasia), corpus callosum dysgenesis, and brainstem asymmetric dysplasia. Exome sequencing showed that all four patients had a novel variant (c.1597C>T:p.Leu533Phe) on the KIF4A gene on chromosome X. We discuss how this variant is possibly pathogenic and could explain the reported phenotype.
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