良性成人家族性肌阵挛性癫痫的脑电图特征。

Electroencephalographic features of benign adult familial myoclonic epilepsy.

机构信息

Department of Neurology, University of Occupational and Environmental Health School of Medicine, Kitakyushu, Japan.

出版信息

Clin Neurophysiol. 2014 Feb;125(2):250-4. doi: 10.1016/j.clinph.2013.08.002. Epub 2013 Sep 4.

DOI:10.1016/j.clinph.2013.08.002

PMID:24011985

Abstract

OBJECTIVE

To investigate electroencephalographic (EEG) features of benign adult familial myoclonic epilepsy (BAFME).

METHODS

We reviewed interictal EEG features in patients with BAFME treated between April 2005 and November 2012 at a tertiary referral center. The diagnostic criteria for BAFME were the presence of infrequent generalized tonic-clonic seizures, myoclonus or myoclonic seizures, and autosomal dominant inheritance. Interictal EEG findings of epilepsy with generalized tonic-clonic seizure only (EGTCS) were reviewed for comparison. We randomly selected 10 generalized spike/polyspike and wave complexes (GSW) for each BAFME patient and measured the duration of them. Photic stimulation and hyperventilation were performed in all.

RESULTS

Nineteen (eight men, 11 women) patients with BAFME were included in this study. The mean frequency of GSW was 4.3±1.0Hz (mean±SD, n=14) in BAFME and 3.2±0.8Hz (n=10) in EGTCS. There was a statistically significant difference (p=0.008) between the two. Photoparoxysmal responses (PPR) were noted in 18 (95%) patients with BAFME but 1 (10%) with EGTCS.

CONCLUSION

Faster frequency of GSW, compared with that in EGTCS, accompanied by PPR may be characteristic EEG features of BAFME.

SIGNIFICANCE

These findings may lead the diagnosis of BAFME.

摘要

目的

研究良性成人家族性肌阵挛癫痫（BAFME）的脑电图（EEG）特征。

方法

我们回顾了 2005 年 4 月至 2012 年 11 月在一家三级转诊中心治疗的 BAFME 患者的发作间期 EEG 特征。BAFME 的诊断标准为存在偶发性全面强直阵挛发作、肌阵挛或肌阵挛发作，以及常染色体显性遗传。为了比较，我们还回顾了仅伴全面强直阵挛发作的癫痫（EGTCS）的发作间期 EEG 发现。我们随机选择了每个 BAFME 患者的 10 个广义棘波/多棘波复合波（GSW），并测量了它们的持续时间。所有患者均进行了光刺激和过度通气。

结果

本研究纳入了 19 例（8 名男性，11 名女性）BAFME 患者。BAFME 患者的 GSW 平均频率为 4.3±1.0Hz（n=14），EGTCS 患者为 3.2±0.8Hz（n=10）。两者之间存在统计学差异（p=0.008）。18 例（95%）BAFME 患者出现光惊反射（PPR），而 1 例（10%）EGTCS 患者出现 PPR。