Get 20% Off Journals at LWW.com

Cognitive impairment and behavioral disorders in Encephalopathy related to Status Epilepticus during slow Sleep: diagnostic assessment and outcome.

Epileptic Disord 2019 Jun;21(S1):71-75

UCL-Great Ormond Street Institute of Child Health, Great Ormond Street Hospital for Children NHS Trust, Member of the European Reference Network EpiCARE, London, UK.

Encephalopathy related to Status Epilepticus during slow Sleep (ESES) is an age-dependent phenomenon, with usual spontaneous resolution during teenage years. However, cognitive outcome is often more disappointing, with permanent cognitive deficits in the large majority of children seen in later life. Presuming this to be an epileptic encephalopathy, current treatment practices are almost exclusively guided by the effect of the AEDs used on the degree of EEG abnormality in sleep. However, the major goal of therapy in ESES syndrome should in fact be to prevent or reduce associated cognitive and neurodevelopmental deficits. Whether or not the EEG pattern of ESES should be completely suppressed to improve cognition is unknown. Discussions on both diagnostic assessment and outcome of cognitive impairment and behavioral disorders should systematically take into account the complexity of the disorder; not only in terms of the evolution or fluctuations of the EEG patterns but also in relation to the underlying etiologies (at least lesional versus non-lesional) and age at diagnosis. We present a common basic assessment protocol, including the minimum technical requirements for polygraphic recording, and a treatment practice protocol that could both be applied in all centres dealing with this rare form of epilepsy. Such an approach would also allow a comprehensive collection of data prospectively, for a better understanding of the natural evolution of the disorder and an evidence-based evaluation of our practices.

Download full-text PDF

Source
http://dx.doi.org/10.1684/epd.2019.1060DOI Listing
June 2019
Save 15% Survey

Similar Publications

Evaluation of adenosine A1 receptor agonists as neuroprotective countermeasures against Soman intoxication in rats.

Toxicol Appl Pharmacol 2021 Feb 22:115466. Epub 2021 Feb 22.

U.S. Army Medical Research Institute of Chemical Defense, Aberdeen Proving Ground, Aberdeen, MD 21010-5400, United States of America. Electronic address:

Soman, an organophosphorus (OP) compound, disrupts nervous system function through inactivation of acetylcholinesterase (AChE), the enzyme that breaks down acetylcholine at synapses. Left untreated, a state of prolonged seizure activity (status epilepticus, SE) is induced, causing widespread neuronal damage and associated cognitive and behavioral impairments. Previous research demonstrated that therapeutic stimulation of A1 adenosine receptors (A1ARs) can prevent or terminate soman-induced seizure. Read More

View Article and Full-Text PDF
February 2021

Longitudinal changes in gray and white matter microstructure during epileptogenesis in pilocarpine-induced epileptic rats.

Seizure 2021 Feb 13. Epub 2021 Feb 13.

Departamento de Neurobiologia Conductual y Cognitiva, Instituto de Neurobiologia, Universidad Nacional Autonoma de Mexico, Campus UNAM-Juriquilla, 76230, Queretaro, Mexico. Electronic address:

Purpose: Temporal lobe epilepsy is associated with tissue abnormalities of several gray and white matter structures that are reproduced in animal models. Few longitudinal studies have focused on the identification of structural differences during epileptogenesis. The diffusion tensor model is a useful tool for evaluating cell death, gliosis, and axonal plasticity in epileptic subjects. Read More

View Article and Full-Text PDF
February 2021

Acute withdrawal of new-generation antiepileptic drugs in epilepsy monitoring units: Safety and efficacy.

Epilepsy Behav 2021 Feb 21;117:107846. Epub 2021 Feb 21.

Department of Neurology, School of Medicine, National Yang-Ming University, Taipei, Taiwan; Department of Neurology, Taipei Municipal Gan-Dau Hospital, Taipei, Taiwan.

Introduction: Acute withdrawal of antiepileptic drugs (AEDs) is a safe and effective approach to provoking seizures in order to complete video-electroencephalogram (V-EEG) studies in a timely manner. Previous studies have focused only on withdrawal from conventional AEDs, and the effects of withdrawal from new-generation AEDs have not been extensively studied.

Materials And Methods: This study examined adult patients with drug-resistant epilepsy admitted to an epilepsy monitoring unit between 2015 and 2018. Read More

View Article and Full-Text PDF
February 2021

Valproic Acid as an Adjuvant Treatment for Generalized Convulsive Status Epilepticus in Adults Admitted to Intensive Care Units: Protocol for a Double-Blind, Multicenter Randomized Controlled Trial.

JMIR Res Protoc 2021 Feb 24;10(2):e22511. Epub 2021 Feb 24.

Centre Hospitalier Poissy Saint Germain en Laye, Poissy, France.

Background: Generalized convulsive status epilepticus (GCSE) is a frequent medical emergency. GCSE treatment focuses on the administration of benzodiazepines followed by a second-line antiepileptic drug (AED). Despite this stepwise strategy, GCSE is not controlled in one-quarter of patients and is associated with protracted hospitalization, high mortality, and long-term disability. Read More

View Article and Full-Text PDF
February 2021

Enzyme Replacement Therapy for Succinic Semialdehyde Dehydrogenase Deficiency: Relevance in γ-Aminobutyric Acid Plasticity.

J Child Neurol 2021 Feb 24:883073821993000. Epub 2021 Feb 24.

FM Kirby Neurobiology Center, 1862Boston Children's Hospital, Boston, MA, USA.

Succinic semialdehyde dehydrogenase deficiency (SSADHD) is a rare inborn metabolic disorder caused by the functional impairment of SSADH (encoded by the gene), an enzyme essential for metabolism of the inhibitory neurotransmitter γ-aminobutyric acid (GABA). In SSADHD, pathologic accumulation of GABA and its metabolite γ-hydroxybutyrate (GHB) results in broad spectrum encephalopathy including developmental delay, ataxia, seizures, and a heightened risk of sudden unexpected death in epilepsy (SUDEP). Proof-of-concept systemic SSADH restoration via enzyme replacement therapy increased survival of SSADH knockout mice, suggesting that SSADH restoration might be a viable intervention for SSADHD. Read More

View Article and Full-Text PDF
February 2021