Epilepsy With Auditory Features: From Etiology to Treatment.

Alessandro Furia,Lorenzo Ferri,Stefania Mazzoni,Laura Licchetta,Raffaella Minardi,Barbara Mostacci,Francesca Bisulli,Paolo Tinuper,Veronica Menghi,Lorenzo Muccioli

doi:10.3389/fneur.2021.807939

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.

Highlights

Epilepsy with auditory features (EAF) is a focal epilepsy included in the group of focal syndromes with onset at variable age in the new ILAE Classification, besides focal epilepsy with variable foci (FFEAF) and sleep-related hypermotor epilepsy (SHE), with which it shares some genetic aspects [1]
Developmental brain abnormalities caused by pathogenic variants in DEPDC5 and genes coding for other component of the mTOR pathway might justify its association with focal cortical dysplasia (FCD), which according to its anatomical position might cause a wide array of different DEPDC5-related focal epilepsies
More work should be done on genetics, perhaps shifting the original paradigm from a disease dominated by LGI1 mutations to a multifactorial syndrome that can be stratified in different groups

Summary

Introduction

Epilepsy with auditory features (EAF) is a focal epilepsy included in the group of focal syndromes with onset at variable age in the new ILAE Classification, besides focal epilepsy with variable foci (FFEAF) and sleep-related hypermotor epilepsy (SHE), with which it shares some genetic aspects [1]. EAF phenotype is characterized by seizures with auditory symptoms or aphasia, suggesting a lateral temporal lobe involvement. Of the 11 members affected, 55% reported auditory symptoms: all had focal to bilateral tonic-clonic seizures.

Results

Conclusion