Definition and diagnostic criteria of sleep-related hypermotor epilepsy
Open Access
- 10 May 2016
- journal article
- review article
- Published by Ovid Technologies (Wolters Kluwer Health) in Neurology
- Vol. 86 (19), 1834-1842
- https://doi.org/10.1212/wnl.0000000000002666
Abstract
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.Keywords
This publication has 60 references indexed in Scilit:
- A Tumor Suppressor Complex with GAP Activity for the Rag GTPases That Signal Amino Acid Sufficiency to mTORC1Science, 2013
- Distinctive polysomnographic traits in nocturnal frontal lobe epilepsyEpilepsia, 2012
- Somatic Activation of AKT3 Causes Hemispheric Developmental Brain MalformationsNeuron, 2012
- Neuroethological approach to frontolimbic epileptic seizures and parasomnias: The same central pattern generators for the same behavioursRevue Neurologique, 2009
- The FLEP scale in diagnosing nocturnal frontal lobe epilepsy, NREM and REM parasomnias: Data from a tertiary sleep and epilepsy unitEpilepsia, 2008
- Surgical treatment of drug-resistant nocturnal frontal lobe epilepsyBrain, 2006
- Increased Sensitivity of the Neuronal Nicotinic Receptor α2 Subunit Causes Familial Epilepsy with Nocturnal Wandering and Ictal FearAmerican Journal of Human Genetics, 2006
- Familial Partial Epilepsy with Variable Foci: Clinical Features and Linkage to Chromosome 22q12Epilepsia, 2004
- A de novo mutation in sporadic nocturnal frontal lobe epilepsyAnnals of Neurology, 2000
- Familial partial epilepsy with variable foci: A new partial epilepsy syndrome with suggestion of linkage to chromosome 2Annals of Neurology, 1998