Sleep-related hypermotor epilepsy: prevalence, impact and management strategies

Veronica Menghi,1 Francesca Bisulli,2 Paolo Tinuper,2 Lino Nobili3,4 1Department of Biomedical and Neuromotor Sciences (DIBINEM), University of Bologna, Bologna, Italy; 2IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy; 3“Claudio Munari” Center for Epilepsy Su...

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Autores principales:	Menghi V, Bisulli F, Tinuper P, Nobili L
Formato:	article
Lenguaje:	EN
Publicado:	Dove Medical Press 2018
Materias:	epilepsy parasomnias sleep-related hypermotor epilepsy nocturnal frontal lobe epilepsy epidemiology. Psychiatry RC435-571 Neurophysiology and neuropsychology QP351-495
Acceso en línea:	https://doaj.org/article/74c0752dbe01472c9472ad870111bc78
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spelling	oai:doaj.org-article:74c0752dbe01472c9472ad870111bc782021-12-02T05:50:23ZSleep-related hypermotor epilepsy: prevalence, impact and management strategies1179-1608https://doaj.org/article/74c0752dbe01472c9472ad870111bc782018-10-01T00:00:00Zhttps://www.dovepress.com/sleep-related-hypermotor-epilepsy-prevalence-impact-and-management-str-peer-reviewed-article-NSShttps://doaj.org/toc/1179-1608Veronica Menghi,1 Francesca Bisulli,2 Paolo Tinuper,2 Lino Nobili3,4 1Department of Biomedical and Neuromotor Sciences (DIBINEM), University of Bologna, Bologna, Italy; 2IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy; 3“Claudio Munari” Center for Epilepsy Surgery, Niguarda Hospital, Milan, Italy; 4Department of Neuroscience (DINOGMI), IRCCS, Giannina Gaslini Institute, University of Genoa, Genoa, Italy Abstract: Sleep-related hypermotor epilepsy (SHE), previously called nocturnal frontal lobe epilepsy (NFLE), is a focal epilepsy characterized by asymmetric tonic/dystonic posturing and/or complex hyperkinetic seizures occurring mostly during sleep. SHE fulfills the definition of rare disease with an estimated minimum prevalence of 1.8/100,000 individuals, and it represents about 10% of drug-resistant surgical cases. Although SHE and autosomal-dominant SHE (ADSHE) have been considered benign epileptic conditions for a long time, emerging data have shed light on the severity of this disorder and some peculiar features can impact negatively on the quality of life of SHE patients. In fact, seizure frequency can be very high, resulting in nocturnal sleep fragmentation with possible diurnal consequences such as excessive sleepiness and fatigue. Moreover, recent studies, adopting a systematic neuropsychological assessment, have shown deficits in memory, executive functions and visuo-spatial abilities in almost half of SHE patients. Intellectual disabilities and psychiatric disorders have also been reported in some genetic forms. SHE may also exert a negative effect on health-related quality of life, especially in domains pertaining to a patient’s role in the family, social context and patient’s illness experience.   Despite a good response to pharmacological treatment, especially with carbamazepine, 30% of SHE patients suffer from drug-resistant seizures. Finally, recent studies suggest a poor prognosis in a high percentage of SHE patients with a 20.4% cumulative probability of achieving terminal remission at 10 years from onset. For selected drug-resistant SHE patients, epilepsy surgery is the only treatment offering high probability of recovery, both for seizures and for epilepsy-related sleep alterations. Keywords: epilepsy, parasomnias, sleep-related hypermotor epilepsy, nocturnal frontal lobe epilepsy, epidemiologyMenghi VBisulli FTinuper PNobili LDove Medical Pressarticleepilepsyparasomniassleep-related hypermotor epilepsynocturnal frontal lobe epilepsyepidemiology.PsychiatryRC435-571Neurophysiology and neuropsychologyQP351-495ENNature and Science of Sleep, Vol Volume 10, Pp 317-326 (2018)
institution	DOAJ
collection	DOAJ
language	EN
topic	epilepsy parasomnias sleep-related hypermotor epilepsy nocturnal frontal lobe epilepsy epidemiology. Psychiatry RC435-571 Neurophysiology and neuropsychology QP351-495
spellingShingle	epilepsy parasomnias sleep-related hypermotor epilepsy nocturnal frontal lobe epilepsy epidemiology. Psychiatry RC435-571 Neurophysiology and neuropsychology QP351-495 Menghi V Bisulli F Tinuper P Nobili L Sleep-related hypermotor epilepsy: prevalence, impact and management strategies
description	Veronica Menghi,1 Francesca Bisulli,2 Paolo Tinuper,2 Lino Nobili3,4 1Department of Biomedical and Neuromotor Sciences (DIBINEM), University of Bologna, Bologna, Italy; 2IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy; 3“Claudio Munari” Center for Epilepsy Surgery, Niguarda Hospital, Milan, Italy; 4Department of Neuroscience (DINOGMI), IRCCS, Giannina Gaslini Institute, University of Genoa, Genoa, Italy Abstract: Sleep-related hypermotor epilepsy (SHE), previously called nocturnal frontal lobe epilepsy (NFLE), is a focal epilepsy characterized by asymmetric tonic/dystonic posturing and/or complex hyperkinetic seizures occurring mostly during sleep. SHE fulfills the definition of rare disease with an estimated minimum prevalence of 1.8/100,000 individuals, and it represents about 10% of drug-resistant surgical cases. Although SHE and autosomal-dominant SHE (ADSHE) have been considered benign epileptic conditions for a long time, emerging data have shed light on the severity of this disorder and some peculiar features can impact negatively on the quality of life of SHE patients. In fact, seizure frequency can be very high, resulting in nocturnal sleep fragmentation with possible diurnal consequences such as excessive sleepiness and fatigue. Moreover, recent studies, adopting a systematic neuropsychological assessment, have shown deficits in memory, executive functions and visuo-spatial abilities in almost half of SHE patients. Intellectual disabilities and psychiatric disorders have also been reported in some genetic forms. SHE may also exert a negative effect on health-related quality of life, especially in domains pertaining to a patient’s role in the family, social context and patient’s illness experience.   Despite a good response to pharmacological treatment, especially with carbamazepine, 30% of SHE patients suffer from drug-resistant seizures. Finally, recent studies suggest a poor prognosis in a high percentage of SHE patients with a 20.4% cumulative probability of achieving terminal remission at 10 years from onset. For selected drug-resistant SHE patients, epilepsy surgery is the only treatment offering high probability of recovery, both for seizures and for epilepsy-related sleep alterations. Keywords: epilepsy, parasomnias, sleep-related hypermotor epilepsy, nocturnal frontal lobe epilepsy, epidemiology
format	article
author	Menghi V Bisulli F Tinuper P Nobili L
author_facet	Menghi V Bisulli F Tinuper P Nobili L
author_sort	Menghi V
title	Sleep-related hypermotor epilepsy: prevalence, impact and management strategies
title_short	Sleep-related hypermotor epilepsy: prevalence, impact and management strategies
title_full	Sleep-related hypermotor epilepsy: prevalence, impact and management strategies
title_fullStr	Sleep-related hypermotor epilepsy: prevalence, impact and management strategies
title_full_unstemmed	Sleep-related hypermotor epilepsy: prevalence, impact and management strategies
title_sort	sleep-related hypermotor epilepsy: prevalence, impact and management strategies
publisher	Dove Medical Press
publishDate	2018
url	https://doaj.org/article/74c0752dbe01472c9472ad870111bc78
work_keys_str_mv	AT menghiv sleeprelatedhypermotorepilepsyprevalenceimpactandmanagementstrategies AT bisullif sleeprelatedhypermotorepilepsyprevalenceimpactandmanagementstrategies AT tinuperp sleeprelatedhypermotorepilepsyprevalenceimpactandmanagementstrategies AT nobilil sleeprelatedhypermotorepilepsyprevalenceimpactandmanagementstrategies
_version_	1718400236715507712

Sleep-related hypermotor epilepsy: prevalence, impact and management strategies

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