PDF(Pubmed)
Abstract：
Epilepsy with auditory features (EAF) is a focal epilepsy belonging to the focal epileptic syndromes with onset at variable age according to the new ILAE Classification. It is characterized by seizures with auditory aura or receptive aphasia suggesting a lateral temporal lobe involvement of the epileptic discharge. Etiological factors underlying EAF are largely unknown. In the familial cases with an autosomal dominant pattern of inheritance several genes have been involved, among which the first discovered, LGI1, was thought to be predominant. However, increasing evidence now points to a multifactorial etiology, as familial and sporadic EAF share a virtually identical electro-clinical characterization and only a few have a documented genetic etiology. Patients with EAF usually have an unremarkable neurological examination and a good response to antiseizure medications. However, it must be underscored that total remission might be lower than expected and that treatment withdrawal might lead to relapses. Thus, a proper understanding of this condition is in order for better patient treatment and counseling. Further studies are still required to further characterize the many facets of EAF.
摘要：
根据新的ILAE分类，具有听觉特征的癫痫（EAF）是属于局灶性癫痫综合征的局灶性癫痫，其发病年龄可变。它的特征是具有听觉先兆或接受性失语症的癫痫发作，表明癫痫放电的颞叶外侧受累。EAF的病因因素在很大程度上是未知的。在具有常染色体显性遗传模式的家族病例中，涉及几个基因，其中第一个发现的，LGI1被认为是主要的。然而,现在越来越多的证据指向多因素病因，由于家族性和散发性EAF具有几乎相同的电临床特征，并且只有少数具有已记录的遗传病因。EAF患者通常没有明显的神经系统检查，对抗癫痫药物反应良好。然而,必须强调的是,完全缓解可能低于预期,停药治疗可能导致复发.因此，对这种情况的正确理解是为了更好的病人治疗和咨询。仍然需要进一步的研究来进一步表征EAF的许多方面。
