AUTOSOMAL-DOMINANT NOCTURNAL FRONTAL-LOBE EPILEPSY - ELECTROCLINICAL PICTURE

Purpose: Nocturnal frontal lobe epilepsy is a disorder that is difficu lt to diagnose because its clinical presentation is often limited to m otor behavior during sleep. For this reason, a misleading diagnosis of benign nocturnal parasomnias might be possible. Recently, an inherite d form of nocturnal frontal lobe epilepsy was described in some famili es. The aim of our work was to describe the electroclinical pattern of a sample of familial cases with this syndrome. Methods: We observed 3 3 patients, all complaining of frequent nocturnal motor attacks, from eight Italian families. The family trees were strongly supportive of a utosomal dominant inheritance. We performed a full-night video-polysom nographic monitoring in 12 patients. Results: The recordings showed at tacks in all patients, there being a widespread pattern of motor activ ity. Ictal and interictal EEG abnormalities were often hidden and, unl ess associated with a video recording, were of no use for the final di agnosis. Intraindividual stereotypy, abrupt onset, and semiology of at tacks allowed differentiation from healthy subjects' nocturnal motor b ehavior. Conclusions: Autosomal dominant nocturnal frontal lobe epilep sy is probably not uncommon. Full-night video-polysomnographic monitor ing is fundamental for the differential diagnosis of benign parasomnia s and, consequently, for appropriate therapy.