Lennox-Gastaut syndrome in adulthood: Clinical and EEG features
Lennox-Gastaut syndrome in adulthood: Clinical and EEG features
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DOI：
10.1016/j.eplepsyres.2010.01.012
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发表时间：
2010-05-01
影响因子：
2.2
通讯作者：
Genton, Pierre
中科院分区：
文献类型：
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作者：
Ferlazzo, Edoardo;Nikaronova, Marina;Genton, Pierre
目的：我们进行了一项回顾性研究，调查癫痫发作，脑电图，社会和认知的结果在成人LGS subjects.Methods：我们回顾性评估了27例LGS患者，年龄40-59岁。我们评估特别是不同的癫痫发作类型和脑电图结果的演变，以及认知和社会outcome.Results：在疾病的早期阶段，所有患者的强直性发作（TS）在清醒和睡眠，20/27有不典型的缺席（AA），更罕见的其他癫痫发作类型。EEG显示21/27例患者的缓慢背景活动，22/27例患者清醒时出现弥漫性慢棘波放电（DSSW），所有患者睡眠时均出现弥漫性快节奏（DFR）爆发。在最后一次观察时，11例患者仅在清醒时出现TS，但所有患者在睡眠时仍出现TS; 6例患者持续存在AA。EEG显示12/27例患者BA正常，仅7/27例仍存在DSSW。而睡眠脑电图显示DFR持续存在。27例患者中有26例出现中度至重度认知功能障碍。结论：成年LGS患者睡眠期TS仍是主要发作类型，而且标准清醒EEG可能正常。因此，多导睡眠图也是成人LGS患者最重要的研究手段。(C)2010 Elsevier B. V.保留所有权利。
Purpose: We performed a retrospective study to investigate seizure, EEG, social and cognitive outcome in adult LGS subjects.Methods: We retrospectively evaluated 27 LGS patients aged 40-59 years. We assessed in particular the evolution of different seizure types and EEG findings, as well as cognitive and social outcome.Results: During the early stages of the disease, all patients presented tonic seizures (TS) during wakefulness and sleep, 20/27 had atypical absences (AA), more rarely other seizure types. EEG showed slow background activity in 21/27 patients, diffuse slow spike-wave discharges (DSSW) during wakefulness in 22/27, and bursts of diffuse fast rhythms (DFR) in sleep in all patients. At last observation, 11 patients only had TS during wakefulness, but all still presented TS during sleep; AA persisted in 6 patients. EEG showed normal BA in 12/27 patients; only 7/27 still presented DSSW. On the contrary, sleep EEG showed the persistence of DFR in all. A moderate to severe cognitive impairment was observed in 26/27 patients.Conclusions: In adult LGS patients TS during sleep remain the major seizure type; moreover, a standard waking EEG may be normal. Thus, polysomnography represents the most important mean of investigation also in adult LGS patients. (C) 2010 Elsevier B.V. All rights reserved.