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婴儿痉挛症、Lennox-Gastaut综合征及进行性肌阵挛癫痫的诊断

Diagnosis of infantile spasms, Lennox-Gastaut syndrome, and progressive myoclonic epilepsy.

作者信息

Shields W Donald

机构信息

Division of Pediatric Neurology, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095, U.S.A.

出版信息

Epilepsia. 2004;45 Suppl 5:2-4. doi: 10.1111/j.0013-9580.2004.05002.x.

Abstract

The epilepsies of childhood are distinguished by an interesting dichotomy between the benign and catastrophic disorders. Approximately 50% of children outgrow childhood epilepsy as they mature; although the disorder is disruptive for children and families alike, it is not considered a medical disaster. The catastrophic epilepsies of childhood, in contrast, are associated with significant morbidity and mortality. Infantile spasms, Lennox-Gastaut syndrome, and the progressive myoclonic epilepsies are correlated with significant disability and a multiplicity of underlying etiologies. Accurate diagnosis of both the syndrome and the etiology is very important for treatment purposes, as well as for family education, since many of the disorders have a significant genetic component.

摘要

儿童癫痫的特点是在良性和灾难性疾病之间存在一种有趣的二分法。大约50%的儿童在成长过程中会摆脱儿童癫痫;尽管这种疾病对儿童及其家庭都会造成干扰,但它并不被视为医疗灾难。相比之下,儿童灾难性癫痫则与显著的发病率和死亡率相关。婴儿痉挛症、伦诺克斯-加斯东综合征和进行性肌阵挛癫痫与严重残疾和多种潜在病因相关。准确诊断综合征和病因对于治疗以及家庭教育都非常重要,因为许多疾病都有显著的遗传成分。

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