Frontal lobe dysfunctions in patients with juvenile myoclonic epilepsy

Abstract

Juvenile myoclonic epilepsy (JME) is a common form of idiopathic generalized epilepsy. Behavioral and cognitive problems are frequently observed in JME. Although the underlying mechanisms are still unknown, recent neuropsychological and neuroimaging studies provide strong evidence for frontal lobe dysfunction. Neuropsychological studies have shown JME patients to be impaired in a number of executive functions such as concept formation, abstract reasoning, mental flexibility, and cognitive speed and planning. They scored below average on measures of attention, immediate verbal memory, working memory, and verbal fluency. The patients with JME performed worse than the group with temporal lobe epilepsy on some frontal tasks. Some specific personality features, such as unreliability and emotional instability, poor social adjustment, high rates of anxiety, mood disorders, phobias and somatization disorders seen in patients with JME have also been described in patients with frontal lobe-lesions. Quantitative magnetic resonance imaging studies have shown regional, frontal cortical and thalamic grey matter abnormalities. These findings support the pathophysiological concept of functional abnormalities in the thalamo-cortical circuit in JME. The additional impact of interictal epileptiform electroencephalography activity on cognitive functions was suggested. Poor working memory and impaired executive and motor performance in JME patients may also be attributed to altered dopamine signaling in the striatum and frontal lobes. JME may be a non-uniform condition. Different genetic and pathophysiologic mechanisms could underlie the clinically observed differences in prognosis, cognitive functioning, personality traits, and frontal lobe deficits.

Terminal remission is possible in some patients with juvenile myoclonic epilepsy without therapy

“…Family history of epilepsy, age at onset of seizures, sex, presence of PPR, results of conventional magnetic resonance imaging (cMRI) and delayed diagnosis were not significantly associated with drug resistance as reported in some previous studies [15,16]. Contrary to our results, the true-resistant course was significantly associated with psychiatric disorders [14][15][16] and the presence of thyroid diseases [16]. Myoclonic jerks alone, without GTCS or absence seizures have been reported to occur in 7-17% of JME probands [1].…”

“…It is usually assumed that continuous pharmacotherapy is required, although about 10% of them appear to have permanent remission in adolescence [13]. Discontinuing AED treatment in JME is very important for the long-term social outcome and quality of life of the patients and requires an individual risk assessment [9,14].…”

Cognitive Dysfunction in Juvenile Myoclonic Epilepsy (JME) – A Tertiary Care Center Study

“…The results pertaining to both FAB and EXIT were in agreement with the previous studies reiterating the significant executive dysfunction in JME patients. [ 6 7 8 9 17 ]…”

“…Various studies employing sensitive neuropsychological tests have demonstrated frontal lobe dysfunction in patients with JME. [ 6 7 8 9 ]…”