lenox - gastaut综合征的诊断和年龄相关演变。不同患者年龄阶段的管理

Q4 Medicine
E. Belousova, S. Burd, N. A. Ermolenko, K. Mukhin
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引用次数: 0

摘要

lenox - gastaut综合征是一种儿童期发病的癫痫性脑病。经典的三重诊断标准是众所周知的:1)存在各种类型的难治性癫痫发作(强直性,非典型缺席,肌阵挛性,强直性/无强直性下降发作,全身性强直性-阵挛性,局灶性);2)认知障碍伴频繁的行为障碍(癫痫发作开始时并不总是明显的);3)睡眠脑电图显示清醒时的全身性慢(≤2.5 Hz)峰波活动和全身性阵发性快活动。尽管癫痫发作通常发生在8岁之前(高峰在3-5岁),但lenox - gastaut综合征通常具有终生病程。许多患有这种综合征的患者在成年后患有难治性癫痫,然而,并不总是提供适当的综合征诊断。扩大诊断lenox - gastaut综合征的标准将允许选择适当的治疗算法。鲁非胺是lenox - gastaut综合征相关癫痫发作辅助治疗的第二选择药物。然而,lenox - gastaut综合征患者从儿科到成人的临床转变可能会带来一些障碍。因此,有效的患者管理不仅需要控制癫痫发作,还需要通过影响认知和行为问题、睡眠障碍、残疾(身体和社会)、教育问题和就业来改善患者的生活质量。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Diagnostics and age-related evolution of Lennox–Gastaut syndrome. Management in diverse patient age periods
Lennox–Gastaut syndrome is an epileptic encephalopathy with onset in childhood. The classical triad of diagnostic criteria is well known: 1) presence of various types of refractory epileptic seizures (tonic, atypical absences, myoclonic, tonic/atonic drop attacks, generalized tonic-clonic, focal); 2) cognitive disorders with frequent behavioral disorders (not always evident by the beginning of epileptic seizures); 3) generalized, slow (≤2.5 Hz) spike-wave activity of wakefulness and generalized paroxysmal fast activity on sleep electroencephalogram. Despite the seizure onset usually occurring before the age of 8 (peak at 3–5) years old, the Lennox–Gastaut syndrome is often featured with a lifelong course. Many patients with this syndrome suffer from refractory epilepsy in adulthood, however, not always being provided a proper syndromological diagnosis. Expanding the criteria to diagnose the Lennox–Gastaut syndrome discussed here would allow to choose a proper treatment algorithm. Rufinamide is the drug of the second choice in the adjunctive therapy of epileptic seizures associated with Lennox–Gastaut syndrome. However, a pediatric-to-adult clinic transition of patients with Lennox–Gastaut syndrome may pose some obstacles. Herein, an effective patient management requires not only seizure control, but also improvement of patient's quality of life by influencing cognitive and behavioral issues, sleep disorders, disability (both physical and social), educational problems and employment.
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来源期刊
Epilepsy and Paroxysmal Conditions
Epilepsy and Paroxysmal Conditions Medicine-Neurology (clinical)
CiteScore
0.90
自引率
0.00%
发文量
31
审稿时长
8 weeks
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