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Idiopathic encephalopathy related to status epilepticus during slow sleep (ESES) as a "pure" model of epileptic encephalopathy. An electroclinical, genetic, and follow-up study.
Epilepsy Behav. 2019 08; 97:244-252.EB

Abstract

OBJECTIVE

The objective of the study was to investigate electroclinical and neuropsychological features, genetic background, and evolution of children with idiopathic encephalopathy with status epilepticus during slow sleep (ESES), including Landau-Kleffner syndrome (LKS).

MATERIAL AND METHODS

All children diagnosed with idiopathic ESES at the Danish Epilepsy Centre between March 2003 and December 2014 were retrospectively reviewed. Repeated 24-hour electroencephalography (24-h EEG) recordings, neuropsychological assessments, and clinical-neurological evaluation were performed throughout the follow-up in all patients. In 13 children, genetic investigations were performed.

RESULTS

We collected 24 children (14 males and 10 females). Mean age at ESES diagnosis was 6 years, and mean ESES duration was 2 years and 7 months. Twenty-one children had epileptic seizures. Three children had LKS. Topography of sleep-related EEG epileptic abnormalities was diffuse in 3 subjects, hemispheric in 6, multifocal in 9, and focal in 6. During the active phase of ESES, all children presented with a heterogeneous combination of behavioral and cognitive disturbances. In 14 children, a parallel between severity of the clinical picture and spike-wave index (SWI) was observed. We could not find a strict correlation between the type and severity of neurobehavioral impairment and the side/topography of sleep-related EEG discharges during the active phase of ESES. At the last follow-up, 21 children were in remission from ESES. Complete recovery from neurobehavioral disorders was observed in 5 children. Genetic assessment, performed in 13 children, showed GRIN2A variant in two (15.4%).

SIGNIFICANCE

Our patients with idiopathic ESES showed a heterogeneous pattern of epileptic seizures, neurobehavioral disorders, and sleep EEG features. Only one-fourth of children completely recovered from the neuropsychological disturbances after ESES remission. Lack of correlation between severity/type of cognitive derangement and SWI and/or topography of sleep EEG epileptic abnormalities may suggest the contribution of additional factors (including impaired sleep homeostasis due to epileptic activity) in the neurobehavioral derangement that characterize ESES.

Authors+Show Affiliations

Danish Epilepsy Centre - Filadelfia, Dianalund, Denmark; Child Neuropsychiatry Unit, Department of Neuroscience, University of Parma, Parma, Italy; Child Neuropsychiatry Service of Carpi, Mental Health Department, AUSL Modena, Carpi, Italy. Electronic address: e.pavlidis@ausl.mo.it.Danish Epilepsy Centre - Filadelfia, Dianalund, Denmark; Department of Regional Health Research, University of Southern Denmark, Odense, Denmark.Danish Epilepsy Centre - Filadelfia, Dianalund, Denmark; Department of Regional Health Research, University of Southern Denmark, Odense, Denmark.Danish Epilepsy Centre - Filadelfia, Dianalund, Denmark.Danish Epilepsy Centre - Filadelfia, Dianalund, Denmark.Danish Epilepsy Centre - Filadelfia, Dianalund, Denmark; Department of Clinical Neurophysiology, Aarhus University Hospital, Aarhus, Denmark; Department of Clinical Medicine, Aarhus University, Aarhus, Denmark.University of Bologna, Bologna, Italy.Danish Epilepsy Centre - Filadelfia, Dianalund, Denmark; Institute of Clinical Medicine, University of Copenhagen, Copenhagen, Denmark. Electronic address: guru@filadelfia.dk.Danish Epilepsy Centre - Filadelfia, Dianalund, Denmark; Department of Regional Health Research, University of Southern Denmark, Odense, Denmark.

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

31254844

Citation

Pavlidis, Elena, et al. "Idiopathic Encephalopathy Related to Status Epilepticus During Slow Sleep (ESES) as a "pure" Model of Epileptic Encephalopathy. an Electroclinical, Genetic, and Follow-up Study." Epilepsy & Behavior : E&B, vol. 97, 2019, pp. 244-252.
Pavlidis E, Møller RS, Nikanorova M, et al. Idiopathic encephalopathy related to status epilepticus during slow sleep (ESES) as a "pure" model of epileptic encephalopathy. An electroclinical, genetic, and follow-up study. Epilepsy Behav. 2019;97:244-252.
Pavlidis, E., Møller, R. S., Nikanorova, M., Kölmel, M. S., Stendevad, P., Beniczky, S., Tassinari, C. A., Rubboli, G., & Gardella, E. (2019). Idiopathic encephalopathy related to status epilepticus during slow sleep (ESES) as a "pure" model of epileptic encephalopathy. An electroclinical, genetic, and follow-up study. Epilepsy & Behavior : E&B, 97, 244-252. https://doi.org/10.1016/j.yebeh.2019.05.030
Pavlidis E, et al. Idiopathic Encephalopathy Related to Status Epilepticus During Slow Sleep (ESES) as a "pure" Model of Epileptic Encephalopathy. an Electroclinical, Genetic, and Follow-up Study. Epilepsy Behav. 2019;97:244-252. PubMed PMID: 31254844.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Idiopathic encephalopathy related to status epilepticus during slow sleep (ESES) as a "pure" model of epileptic encephalopathy. An electroclinical, genetic, and follow-up study. AU - Pavlidis,Elena, AU - Møller,Rikke S, AU - Nikanorova,Marina, AU - Kölmel,Margarethe Sophie, AU - Stendevad,Pia, AU - Beniczky,Sandor, AU - Tassinari,Carlo Alberto, AU - Rubboli,Guido, AU - Gardella,Elena, Y1 - 2019/06/26/ PY - 2019/04/23/received PY - 2019/05/20/revised PY - 2019/05/21/accepted PY - 2019/6/30/pubmed PY - 2020/7/3/medline PY - 2019/6/30/entrez KW - ESES KW - Epileptic encephalopathy KW - Sleep homeostasis KW - Slow sleep KW - Status epilepticus SP - 244 EP - 252 JF - Epilepsy & behavior : E&B JO - Epilepsy Behav VL - 97 N2 - OBJECTIVE: The objective of the study was to investigate electroclinical and neuropsychological features, genetic background, and evolution of children with idiopathic encephalopathy with status epilepticus during slow sleep (ESES), including Landau-Kleffner syndrome (LKS). MATERIAL AND METHODS: All children diagnosed with idiopathic ESES at the Danish Epilepsy Centre between March 2003 and December 2014 were retrospectively reviewed. Repeated 24-hour electroencephalography (24-h EEG) recordings, neuropsychological assessments, and clinical-neurological evaluation were performed throughout the follow-up in all patients. In 13 children, genetic investigations were performed. RESULTS: We collected 24 children (14 males and 10 females). Mean age at ESES diagnosis was 6 years, and mean ESES duration was 2 years and 7 months. Twenty-one children had epileptic seizures. Three children had LKS. Topography of sleep-related EEG epileptic abnormalities was diffuse in 3 subjects, hemispheric in 6, multifocal in 9, and focal in 6. During the active phase of ESES, all children presented with a heterogeneous combination of behavioral and cognitive disturbances. In 14 children, a parallel between severity of the clinical picture and spike-wave index (SWI) was observed. We could not find a strict correlation between the type and severity of neurobehavioral impairment and the side/topography of sleep-related EEG discharges during the active phase of ESES. At the last follow-up, 21 children were in remission from ESES. Complete recovery from neurobehavioral disorders was observed in 5 children. Genetic assessment, performed in 13 children, showed GRIN2A variant in two (15.4%). SIGNIFICANCE: Our patients with idiopathic ESES showed a heterogeneous pattern of epileptic seizures, neurobehavioral disorders, and sleep EEG features. Only one-fourth of children completely recovered from the neuropsychological disturbances after ESES remission. Lack of correlation between severity/type of cognitive derangement and SWI and/or topography of sleep EEG epileptic abnormalities may suggest the contribution of additional factors (including impaired sleep homeostasis due to epileptic activity) in the neurobehavioral derangement that characterize ESES. SN - 1525-5069 UR - https://www.unboundmedicine.com/medline/citation/31254844/Idiopathic_encephalopathy_related_to_status_epilepticus_during_slow_sleep__ESES__as_a_"pure"_model_of_epileptic_encephalopathy__An_electroclinical_genetic_and_follow_up_study_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1525-5050(19)30374-9 DB - PRIME DP - Unbound Medicine ER -