![]() ![]() Seizure types in NFLE were classified as follows 1, 10: paroxysmal arousal (PA), hyperkinetic (HK), tonic/ dystonic (TD), or epileptic nocturnal wandering (ENW). The familial form was ascertained when at least one first- or second-degree relative had a history of epilepsy (including NFLE). According to family history, cases were classified as familial or sporadic. Etiology of NFLE was classified as lesional (structural abnormalities at neuroimaging) or nonlesional (no factor of increased risk of seizures identified). * From other outpatient epilepsy offices of the city of Bologna.įollowing the Guidelines for Epidemiologic Studies on Epilepsy, 9 patients were classified as having (1) active epilepsy (at least one epileptic seizure in the previous 5 y, regardless of antiepileptic drug treatment), or (2) epilepsy in remission with treatment (no seizures for ≥ 5 y with AED treatment at the time of ascertainment), or (3) epilepsy in remission without treatment (no seizures for ≥ 5 y without AED treatment at the time of ascertainment). Data refer to the prevalence day (December 31, 2010). Clinical records of subjects identified through sources other than the two principal centers (see flowchart in Figure 1) were reviewed by experts at the Epilepsy Center of Bologna, in order to check the fulfillment of inclusion criteria.įlow diagram of eligible and included patients with nocturnal frontal lobe epilepsy (NFLE). The agreement required for the final diagnosis was 100% otherwise, these cases were considered doubtful and excluded. All cases with videopolysomnographic recording of stereotyped paroxysmal arousals not associated with hypermotor events were carefully reviewed and discussed collegially also considering the follow-up data. The final diagnosis was confirmed by at least two experts in sleep medicine and epileptology (in each area: Epilepsy Center of Modena, Epilepsy Center of Bologna) considering, in addition to ictal semiology from videopolysomnographic recordings, all clinical features useful to discriminate NFLE from parasomnias (such as age at onset, duration, frequency and number of events per night, stage of sleep when events arise, recall on awakening, presence of daytime sleepiness, outcome, and response to treatment). Videopolysomnographic recording of at least one hypermotor episode (either asymmetric tonic-dystonic or hyperkinetic, i.e., bilateral asymmetric tonic/dystonic posturing or other choreoathetoid and ballistic movements of the limbs lasting about 20–30 sec) or at least two minor stereotyped episodes (i.e., paroxysmal arousals).Īll patients underwent a comprehensive clinical, neuro-physiological, and neuroradiological evaluation. This study aimed to (1) estimate the prevalence of NLFE in adults of two areas of the Emilia-Romagna region (the city of Bologna and the province of Modena)-with similar population size but different levels of urbanization and health care organization-to test the consistency of results and (2) to describe the clinical features of NFLE from a population-based perspective. 5, 6 The ratio between sporadic and familial forms is unknown. The nonlesional form of NFLE seems to be predominant, 2 and some familial cases (autosomal dominant NFLE, ADNFLE) 3, 4 are linked to mutations in the genes coding for the subunits of the neuronal acetylcholine receptors (nAChR), and mutations in new genes ( KCNT1, DEPDC). 1 NFLE is reported to be a rare disorder but no epidemiologic data are available. Ictal manifestations are characterized by bizarre motor behavior or sustained asymmetric dystonic posture suggesting frontal lobe involvement. Nocturnal frontal lobe epilepsy (NFLE) is a peculiar form of focal epilepsy in which seizures occur almost exclusively during sleep.
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