The syndrome known as nocturnal frontal lobe epilepsy is recognized worldwide and has been studied in a wide range of clinical and scientific settings (epilepsy, sleep medicine, neurosurgery, pediatric neurology, epidemiology, genetics). Though uncommon, it is of considerable interest to practicing neurologists because of complexity in differential diagnosis from more common, benign sleep disorders such as parasomnias, or other disorders like psychogenic nonepileptic seizures. Moreover, misdiagnosis can have substantial adverse consequences on patients' lives. At present, there is no consensus definition of this disorder and disagreement persists about its core electroclinical features and the spectrum of etiologies involved. To improve the definition of the disorder and establish diagnostic criteria with levels of certainty, a consensus conference using formal recommended methodology was held in Bologna in September 2014. It was recommended that the name be changed to sleep-related hypermotor epilepsy (SHE), reflecting evidence that the attacks are associated with sleep rather than time of day, the seizures may arise from extrafrontal sites, and the motor aspects of the seizures are characteristic. The etiology may be genetic or due to structural pathology, but in most cases remains unknown. Diagnostic criteria were developed with 3 levels of certainty: witnessed (possible) SHE, video-documented (clinical) SHE, and video-EEG-documented (confirmed) SHE. The main research gaps involve epidemiology, pathophysiology, treatment, and prognosis.

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SUMMARYPurpose: Retrospective observations disclosed an overlap between parasomnias and nocturnal frontal lobe epilepsy (NFLE) not only in patients but also in their relatives, suggesting a possible common pathogenetic mechanism. This study aimed to verify whether relatives of patients with NFLE have a higher frequency of parasomnias, namely arousal disorders, and thereby shed light on the still unknown pathophysiologic mechanisms underlying NFLE. Methods: We undertook a case-control family study in which we recruited NFLE probands and healthy controls, matched for age, sex, education, and geographic origin. At least four relatives were included for each proband and control. Each subject underwent a standardized interview, with application of the International Classification of Sleep Disorders-Revised (ICSD-R 2001) minimal criteria to diagnose the lifetime prevalence of the main parasomnias. Results: Four hundred fifty-eight individuals were recruited: 33 NFLE probands, 200 relatives of probands, 31 controls, and 194 control relatives. All NFLE probands but one have sporadic NFLE. The lifetime prevalence of the following parasomnias differed in proband relatives versus control relatives: arousal disorders [odds ratio (OR) 4.7, 95% confidence interval (CI) 2.0-11.6; p < 0.001] and nightmares (OR 2.6, 95% CI 1.6-4.2; p < 0.001) were more frequent among NFLE proband relatives. In the secondary analysis comparing NFLE probands to controls, arousal disorders (OR 6.3, 95% CI 1.3-31.7; p = 0.023) and bruxism (OR 5.4, 95% CI 1.3-21.7; p = 0.017) were more frequent among NFLE probands. Discussion: The higher frequency of arousal disorders in NFLE families suggests an intrinsic link between parasomnias and NFLE and an abnormal (possibly cholinergic) arousal system as a common pathophysiologic mechanism.

Summary: Purpose: To describe the semiological features of auditory aura and to assess their possible lateralizing value in partial epilepsy.Methods: Out of a series of 8,000 patients with epilepsy, we investigated 121 cases with partial seizures in whom auditory features were the first ictal symptom. According to the dominant type of aura, patients were divided into four subgroups-1A (67 cases), 1B (22 cases), 2A (14 cases), and 2B (18 cases)-corresponding to the presence of simple or complex hallucinations and positive or negative illusions, respectively. The side of the epileptic zone (EZ) was defined based on available data: surgical/presurgical study or presence of a neuroradiological lesion, corresponding interictal epileptiform EEG and ictal semiology (level 1); a left EZ was also hypothesized in right-handed patients with ictal aphasia plus a left neuroradiological lesion or a left interictal EEG focus (level 2).Results: Forty-five patients (37%) described the aura as unilateral. The side of epileptogenic zone (EZ) was definable in 36 patients (level 1: 24; level 2: 12). Overall, a unilateral auditory aura was contralateral to the EZ in half of the cases (8/16), but always contralateral in patients studied for presurgical evaluation (4/4). Simple hallucinations lateralized seizure onset on the right side in nine cases, on the left in 12. Among 1B patients (either musical and verbal contents), the EZ was on the left side in all cases (5/5). Positive illusions were associated with right foci in two cases, and left foci in two. Negative illusions always lateralized seizure onset to the dominant hemisphere (6/6).Conclusions: Auditory aura is a rare symptom in partial epilepsy. The perception of the auditory sensation referred to one ear is not a unique lateralizing sign for the contralateral temporal neocortex. Complex hallucinations with verbal content and negative illusion may lateralize seizure onset in the dominant hemisphere. The role of laterality for musical hallucinations remains unclear as it depends on individual musical ability and hemispheric dominance for music.