Definition and diagnostic criteria of sleep-related hypermotor epilepsy

Paolo Tinuper, Francesca Bisulli, J. H. Cross, Dale C. Hesdorffer, Philippe Kahane, Lino Nobili, Federica Provini, Ingrid E. Scheffer, Laura Tassi, Luca Vignatelli, Claudio L. Bassetti, F. Cirignotta, Christopher Derry, Antonio Gambardella, Renzo Guerrini, P. Halasz, Laura Licchetta, Mark Mahowald, Raffaele Manni, Carla MariniBarbara Mostacci, Ilaria Naldi, L. Parrino, Fabienne Picard, Maura Pugliatti, Philippe Ryvlin, Federico Vigevano, Marco Zucconi, Samuel F. Berkovic, Ruth Ottman

Research output: Contribution to journalReview articlepeer-review


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. Themain research gaps involve epidemiology, pathophysiology, treatment, and prognosis.

Original languageEnglish
Pages (from-to)1834-1842
Number of pages9
Issue number19
Publication statusPublished - May 10 2016

ASJC Scopus subject areas

  • Medicine(all)
  • Clinical Neurology


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