Valenti Maria Paola, Rudolf Gabrielle, Carré Sophie, Vrielynck Pascal, Thibault Anne, Szepetowski Pierre, Hirsch Edouard
Department of Neurology, Strasbourg, France.
Epilepsia. 2006 Apr;47(4):766-72. doi: 10.1111/j.1528-1167.2006.00517.x.
Language-induced epilepsy involves seizure precipitation by speaking, reading, and writing. Seizures are similar to those of reading epilepsy (RE). The nosologic position of language-induced epilepsy is not clear. We performed a clinical and neurophysiological study in a multigenerational family with the association of idiopathic generalized epilepsy (IGE) with ictal stuttering as a manifestation of reflex language-induced epilepsy.
Nine members on three generations were studied. All patients underwent video-polygraphic EEG recordings (awake and during sleep). A standardized protocol was applied to test the effect of language and non-language-related tasks.
Six patients presented language-induced jaw jerking that mimicked stuttering and corresponded to focal myoclonus involving facial muscles. This was associated with an IGE phenotype in four of these patients. Focal EEG spikes were found in all six patients by visual analysis and/or back-averaging techniques. The focal spikes were either asymptomatic (when followed by a slow wave) or symptomatic of facial myoclonia (when isolated). Levetiracetam, used as add-on or monotherapy in four patients, suppressed ictal stuttering. One additional case only had a phenotype of IGE without focal features.
This family study demonstrates the phenotypic heterogeneity of the association of IGE phenotype with ictal stuttering (language-related reflex seizure). Our data suggest that this particular form of reflex epilepsy related to language has more similarities with generalized epilepsies than with focal ones. Neurophysiological investigations should be performed more systematically in patients with acquired stuttering, especially if there is family history of IGE.
语言诱发性癫痫是指因说话、阅读和书写而诱发癫痫发作。其发作与阅读性癫痫（RE）相似。语言诱发性癫痫的疾病分类尚不明确。我们对一个多代家族进行了临床和神经生理学研究，该家族中特发性全身性癫痫（IGE）与发作性口吃相关联，发作性口吃是反射性语言诱发性癫痫的一种表现形式。
对三代中的9名成员进行了研究。所有患者均接受了视频多导脑电图记录（清醒和睡眠期间）。应用标准化方案来测试语言和非语言相关任务的影响。
6名患者出现了模仿口吃的语言诱发性下颌抽搐，这与累及面部肌肉的局灶性肌阵挛相对应。其中4名患者伴有IGE表型。通过视觉分析和/或反向平均技术，在所有6名患者中均发现了局灶性脑电图棘波。这些局灶性棘波要么无症状（随后伴有慢波时），要么是面部肌阵挛的症状（孤立出现时）。4名患者使用左乙拉西坦作为附加治疗或单一疗法，抑制了发作性口吃。另外1例仅具有IGE表型，无局灶性特征。
这项家族研究证明了IGE表型与发作性口吃（语言相关反射性发作）关联的表型异质性。我们的数据表明，这种与语言相关的特殊形式的反射性癫痫与全身性癫痫的相似性高于局灶性癫痫。对于获得性口吃患者，尤其是有IGE家族史的患者，应更系统地进行神经生理学检查。
