Definition and diagnostic criteria of sleep-related hypermotor epilepsy

Tinuper, Paolo; Bisulli, Francesca; Cross, J H; Hesdorffer, Dale; Kahane, Philippe; Nobili, Lino; Provini, Federica; Scheffer, Ingrid E; Tassi, Laura; Vignatelli, Luca; Bassetti, Claudio; Cirignotta, Fabio; Derry, Christopher; Gambardella, Antonio; Guerrini, Renzo; Halasz, Peter; Licchetta, Laura; Mahowald, Mark; Manni, Raffaele; Marini, Carla; ... (2016). Definition and diagnostic criteria of sleep-related hypermotor epilepsy. Neurology, 86(19), pp. 1834-1842. Lippincott Williams & Wilkins 10.1212/WNL.0000000000002666

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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.

Item Type:

Journal Article (Review Article)


04 Faculty of Medicine > Department of Head Organs and Neurology (DKNS) > Clinic of Neurology

UniBE Contributor:

Nobili, Lino and Bassetti, Claudio


600 Technology > 610 Medicine & health




Lippincott Williams & Wilkins




Stefanie Hetzenecker

Date Deposited:

28 Jul 2016 12:09

Last Modified:

22 Sep 2017 12:31

Publisher DOI:


PubMed ID:





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