Emotion recognition and social cognition in juvenile myoclonic epilepsy

J Gaggl,L Kronbichler,L Rainer,E Schmid,M Kirschner,E Trinka,M Kronbichler,G Kuchukhidze,J Höfler

doi:10.1007/s10309-019-0261-y

Abstract

Juvenile myoclonic epilepsy (JME) is the most common idiopathic generalized epilepsy. Disease onset is typically in puberty and poor social adjustment and behavioral disturbances, which resemble frontal lobe dysfunction, are often observed. In recent advanced brain imaging studies on JME patients, emotional and behavioral problems have been associated to subtle structural and functional alterations mainly in frontal cortex and thalamus. There is emerging evidence that patients with abnormal emotion processing and regulation, such as those with bipolar disorder, show disrupted connectivity between limbic structures and frontal cortices. There are no neuroimaging or neuropsychological studies related to emotion processing in patients with JME with a focus on limbic structures. We aimed to address the problem of emotional disturbances and social adjustment in JME patients from multiple aspects through thorough functional and structural assessment, which would potentially enable elaboration of a unifying concept explaining neurobiological background of disturbances in emotional processing and social adjustment in JME patients. Results of this study may potentially enable the development of psychological and pharmacological interventional strategies for managing behavioral disturbances in patients with JME.

Highlights

Juvenile myoclonic epilepsy (JME) is one of the most common age-related idiopathic generalized epilepsies with a high genetic predisposition, comprising 5–10% of all epilepsies [1]
We aimed to address the problem of emotional disturbances and social adjustment in JME patients from multiple aspects through thorough functional and structural assessment, which would potentially enable elaboration of a unifying concept explaining neurobiological background of disturbances in emotional processing and social adjustment in JME patients
We aim to compare imaging and neuropsychological data of younger patients with newly manifested JME with those who have longstanding JME in order to determine whether cerebral structural/functional changes are intrinsic to the epileptic condition or whether they represent the result of longstanding seizure activity

Summary

Introduction

Juvenile myoclonic epilepsy (JME) is one of the most common age-related idiopathic generalized epilepsies with a high genetic predisposition, comprising 5–10% of all epilepsies [1]. Patients with JME usually present with massive myoclonic jerks, which comprise the predominant seizure type, often accompanied by generalized tonic-clonic seizures and less often by absences [2]. Despite the fact that standard brain imaging is usually normal in JME patients, volumetric and connectivity magnetic resonance imaging (MRI) studies demonstrated increase of gray matter volume in mesial and basal frontal regions, decreased thalamic volume, and aberrant thalamocortical circuitry [4]. Increased connectivity between the prefrontal cognitive network and motor system is clearly associated with the pathophysiology of JME and explains why the seizures are elicited by cognitive activity [4]

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