The clinical and polysomnographic findings in 100 consecutive cases of nocturnal frontal lobe epilepsy (NFLE) were analysed in a study at the Neurological Institute, University of Bologna, Italy. Males predominated, in a male/female ratio of 7:3. Nocturnal paroxysmal episodes presented at any age from 1 to 64 years (mean 14+/-10 years) but mainly during infancy and adolescence; parasomnias in infancy were followed by NFLE seizures after intervals of 1 to 30 years. A family history of epilepsy or parasomnias occurred in 25% or 39%, respectively. Past histories included sleep disorders (talking, enuresis, head banging, and sleep walking) in early childhood; 7 had birth anoxia, 3 febrile convulsions, and 3 antecedent mild head trauma. CT or MRI abnormalities were found in 14%. EEGs were epileptiform in only 56% of ictal and 50% of interictal sleep records; epileptiform discharges were recorded only by sphenoidal electrodes in 13%.

VideoEEG showed that NFLE is a spectrum of seizure phenomena (paroxysmal arousal from sleep, nocturnal paroxysmal dystonia, and episodic nocturnal wanderings), representing a continuum of different clinical manifestations of a heterogeneous epilepsy syndrome. Carbamazepine controlled the seizures completely in 16% and partially in 48%. [1]

COMMENT. The diagnosis of nocturnal frontal lobe epilepsy (NFLE) should be suspected in infants and children with paroxysmal nocturnal motor episodes during sleep, characterized by clusters of repetitious stereotyped movements that persist into adolescence and are associated with dystonic posturing or agitated behavior. Pelvic thrusting, facial grimacing and moaning are frequent repeated patterns of behavior during seizures. Diurnal episodes include generalized shivering with loss of consciousness, and complaints of tingling and daytime sleepiness. NFLE is often familial and autosomal dominant in inheritance. NFLE responds to treatment with carbamazepine or clonazepam, but seizures almost always recur when therapy is withdrawn.

Benign nocturnal parasomnias - nightmares, night terrors and somnambulism - may be differentiated from NFLE by a different time course, with remission within 7 years, whereas NFLE persists into adolescence. Parasomnias occur infrequently and at irregular intervals, whereas NFLE occurs nightly and repeatedly. Furthermore, parasomnias are not manifested by dystonic posturing or violent agitated motor behavior. The EEG and especially video EEG is diagnostic in approximately 50% of patients. A trial of antiepileptic drugs may sometimes be warranted on clinical grounds, in the absence of EEG confirmation. (See Progress in Pediatric Neurology III. PNB Publ, 1997;pp87-88).