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.
Keywords
Juvenile myoclonic epilepsy - frontal dysfunction
