Idiopathic rolandic epilepsy with "interictal" facial myoclonia and oromotor deficit: A longitudinal EEG and PET study

Purpose: The prognosis of benign epilepsy with centrotemporal spikes (BECTS ) is always favorable as far as the epilepsy is concerned. However, some da ta suggest that affected children may be at risk for minor cognitive impair ment. We report here the longitudinal study of a young girl demonstrating t hat BECTS also may be associated with severe motor disturbances. Methods: BECTS (rare left oromotor seizures, right rolandic spike-waves act ivated during sleep) started at the age of 3 years 6 months in a girl with normal initial psychomotor development. Her clinical, neuropsychological, a nd EEG status was assessed every 3-6 months. Regional cerebral glucose meta bolism was measured by using the [F-18]fluorodeoxyglucose-positron emission tomography (FDG-PET) method. Results: Between the age of 5 and 6 years, the girl had (a) increased seizu re frequency, (b) brief perioral and palpebral myoclonic jerks, concomitant with the spike component of interictal spike-waves, and (c) persistent but fluctuating oromotor deficits (drooling, dysarthria, dysphagia). The EEG s howed a marked increase in abundance and amplitude of wake and sleep interi ctal abnormalities, which became bilateral. Awake FDG-PET revealed a bilate ral increase of glucose metabolism in opercular regions. A complete and def initive EEG and clinical omission occurred at age 5 years 11 months and has persisted since (present age, 7 years 9 months). Conclusions: This case confirms that during BECTS, epileptiform dysfunction s within rolandic areas may induce ''interictal" positive or negative oromo tor symptoms, independent of classic seizures.