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青少年肌阵挛癫痫的临床异质性:超过20年间隔后的随访

Clinical heterogeneity of juvenile myoclonic epilepsy: follow-up after an interval of more than 20 years.

作者信息

Syvertsen Marte R, Thuve Selma, Stordrange Benedicte S, Brodtkorb Eylert

机构信息

Department of Neurology, Drammen Hospital, Drammen, Norway; Department of Neuroscience, Norwegian University of Science and Technology, Trondheim, Norway.

Department of Neuroscience, Norwegian University of Science and Technology, Trondheim, Norway.

出版信息

Seizure. 2014 May;23(5):344-8. doi: 10.1016/j.seizure.2014.01.012. Epub 2014 Jan 23.

DOI:10.1016/j.seizure.2014.01.012
PMID:24512779
Abstract

PURPOSE

The view that juvenile myoclonic epilepsy (JME) is a uniform and life-long disorder is currently being challenged. The aim of this study was to assess the seizure and psychosocial outcome of JME at least 20 years after onset.

METHODS

In 1992, 42 patients with JME were identified. In 2012, 37 agreed to a semi-structured interview. In the remaining five, only medical records were available.

RESULTS

Of 40 patients with known seizure outcome, 21 were in remission for >5 years. Seven were off antiepileptic drugs (AEDs), four being seizure free for >10 years. Myoclonic seizures (MC) evolving to generalized tonic-clonic seizures (GTC) were associated with seizure persistence (p=0.013), whereas >1 year between MC and GTC onset was associated with a trend to GTC remission (p=0.069). Of 19 patients with uncontrolled seizures, eight experienced remission with second generation AEDs. Favorable psychosocial outcome by interview was found in a third, whereas another third had psychiatric comorbidity, seven with substance or alcohol abuse. Psychosocial and seizure outcome did not correlate.

CONCLUSION

This study corroborates the heterogeneity of JME in terms of seizure and psychosocial outcome, but without a clear association between the two. It confirms that seizure control may persist after AED withdrawal in some and supports MC evolving to GTC as a predictor of seizure persistence. Moreover, it suggests that newer broad spectrum AEDs may improve the prognosis of JME; their impact should be focus of prospective studies.

摘要

目的

青少年肌阵挛性癫痫(JME)是一种统一的终身性疾病这一观点目前正受到挑战。本研究的目的是评估JME发病至少20年后的癫痫发作情况及社会心理结局。

方法

1992年,确定了42例JME患者。2012年,37例同意接受半结构化访谈。其余5例仅有病历资料。

结果

在40例已知癫痫发作结局的患者中,21例缓解超过5年。7例停用抗癫痫药物(AEDs),4例无癫痫发作超过10年。肌阵挛发作(MC)演变为全面强直-阵挛发作(GTC)与癫痫发作持续存在相关(p=0.013),而MC与GTC发作间隔超过1年与GTC缓解趋势相关(p=0.069)。在19例癫痫发作未得到控制的患者中,8例使用第二代AEDs后病情缓解。通过访谈发现三分之一患者的社会心理结局良好,而另外三分之一有精神疾病共病,7例存在物质或酒精滥用。社会心理结局与癫痫发作结局不相关。

结论

本研究证实了JME在癫痫发作及社会心理结局方面的异质性,但两者之间无明确关联。它证实了部分患者停用AEDs后癫痫控制仍可持续,并支持MC演变为GTC可作为癫痫发作持续存在的预测指标。此外,提示新型广谱AEDs可能改善JME的预后;其影响应成为前瞻性研究的重点。

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