三名脑损伤患者患韦斯特综合征，病程良性。

West syndrome in three patients with brain injury and a benign course.

作者信息

Russo Angelo, Duchowny Michael, Boni Antonella, Giannotta Melania, Filippini Melissa, Gobbi Giuseppe

机构信息

IRCCS, Institute of Neurological Sciences of Bologna, Child Neurology Unit, via Altura 3, Bologna, Italy.

Department of Neurology and Comprehensive Epilepsy Program, Brain Institute, Nicklaus Children's Hospital, 3200 SW 60th Court, Miami, FL, United States.

出版信息

Epilepsy Behav Case Rep. 2017 Mar 15;8:35-39. doi: 10.1016/j.ebcr.2017.03.002. eCollection 2017.

DOI:10.1016/j.ebcr.2017.03.002

PMID:28794986

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5542381/

Abstract

Infants with West Syndrome and underlying structural pathology typically experience persistent symptomatic focal seizures and intellectual disability. We performed a retrospective case review of 84 patients with West Syndrome evaluated at one institution between 1990 and 2013. From this group we identified three patients with West syndrome and congenital hemiplegia who later developed genetic epilepsy features and had normal intellectual development. This outcome is highly unusual and raises important questions about the relationship and possible influence of genetic epilepsy in patients with pre-existent West Syndrome and brain injury.

摘要

患有韦斯特综合征及潜在结构病理学问题的婴儿通常会经历持续性症状性局灶性癫痫发作和智力残疾。我们对1990年至2013年间在一家机构接受评估的84例韦斯特综合征患者进行了回顾性病例分析。在这个群体中，我们确定了3例患有韦斯特综合征和先天性偏瘫的患者，他们后来出现了遗传性癫痫特征，且智力发育正常。这一结果非常罕见，并引发了关于遗传性癫痫在已有韦斯特综合征和脑损伤患者中的关系及可能影响的重要问题。