Sleep-related hypermotor epilepsy

Abstract

Objective:To assess the long-term outcome of sleep-related hypermotor epilepsy (SHE).Methods:We retrospectively reconstructed a representative cohort of patients diagnosed with SHE according to international diagnostic criteria, sleep-related seizures ≥75% and follow-up ≥5 years. Terminal remission (TR) was defined as a period of ≥5 consecutive years of seizure freedom at the last follow-up. We used Kaplan-Meier estimates to calculate the cumulative time-dependent probability of TR and to generate survival curves. Univariate and multivariate Cox regression analyses were performed.Results:We included 139 patients with a 16-year median follow-up (2,414 person-years). The mean age at onset was 13 ± 10 years. SHE was sporadic in 86% of cases and familial in 14%; 16% of patients had underlying brain abnormalities. Forty-five percent of patients had at least 1 seizure in wakefulness lifetime and 55% had seizures only in sleep (typical SHE). At the last assessment, 31 patients achieved TR (TR group, 22.3%), while 108 (NTR group, 77.7%) still had seizures or had been in remission for <5 years. The cumulative TR rate was 20.4%, 23.5%, and 28.4% by 10, 20, and 30 years from inclusion. At univariate analysis, any underlying brain disorder (any combination of intellectual disability, perinatal insult, pathologic neurologic examination, and brain structural abnormalities) and seizures in wakefulness were more frequent among the NTR group (p = 0.028; p = 0.043). Absence of any underlying brain disorder (hazard ratio 4.21, 95% confidence interval 1.26–14.05, p = 0.020) and typical SHE (hazard ratio 2.76, 95% confidence interval 1.31–5.85, p = 0.008) were associated with TR.Conclusions:Our data show a poor prognosis of SHE after a long-term follow-up. Its outcome is primarily a function of the underlying etiology.

From nocturnal frontal lobe epilepsy to Sleep-Related Hypermotor Epilepsy: A 35-year diagnostic challenge

“…Epilepsy onset is mostly in the second decade of life with a peak incidence during childhood [10,11,9,12], although adult onset has also been reported [10,11,9]. Seizure frequency at onset is usually high and patients generally experience many attacks per night [9,13] although episodes may diminish during adulthood [11,10]. Seizures during wakefulness occasionally occur [9,13].…”

“…Seizure frequency at onset is usually high and patients generally experience many attacks per night [9,13] although episodes may diminish during adulthood [11,10]. Seizures during wakefulness occasionally occur [9,13]. Frequent parasomnias are reported not only by NFLE patients but also by their healthy relatives [14].…”

“…Frequent parasomnias are reported not only by NFLE patients but also by their healthy relatives [14]. Ictal and interictal EEG is unrevealing in a large proportion of cases [9,13] with the sole exception of patients with drug-resistant epilepsy undergoing prolonged video-EEG recordings for surgical purposes [15]. During NFLE seizures (NFLS), ictal EEG is frequently obscured by artifacts caused by the violent movements, making it hard to identify an epileptic discharge.…”

“…In addition, epileptic foci in deeper areas of the brain like the orbitofrontal or mesial structures are difficult to detect with scalp EEG. The limited contribution of scalp EEG to localization and lateralization of the seizure onset zone is accompanied by a low incidence of positive neuroradiological findings [15,13].…”

Disorders of Arousal in adults: new diagnostic tools for clinical practice

“…SHE is sleeprelated focal epilepsy characterized by complex, often bizarre motor behaviors, including asymmetrical tonic or dystonic posture. Differential diagnosis between DOA and SHE is not always easy due to the possible presence of violent behaviors, complex automatisms, ambulation, and vocalizations in both conditions (Provini et al 1999;Tinuper et al 2007;Bisulli et al 2012;Licchetta et al 2017). The occurrence in the first part of the night, the absence of stereotypical and abnormal movements, such as dystonic and dyskinetic postures, the long duration of the episodes, and the low rate of same-night recurrence are DOA key features (Provini et al 1999(Provini et al , 2011Tinuper et al 2007) (Table 2).…”