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Electrical Status Epilepticus in Sleep | Electrical Status Epilepticus in Sleep: Response of Neuropsychological Deficits and Epileptiform Activity to Clobazam Treatment

Electrical Status Epilepticus in Sleep research study

What is the primary objective of this study?

Electrical status epilepticus in sleep (ESES) is a pattern of abnormal discharges in the electroencephalogram (EEG). Children who have this pattern present seizures and neuropsychological regression. There are no studies that systematically evaluate the response of abnormal discharges in the EEG, seizures and neuropsychological regression to different antiepileptic treatments. Therefore, treatment strategies in ESES are not based on scientific evidence. High-dose benzodiazepines such as diazepam (valium) have been reported to improve the severe EEG abnormalities of patients with ESES in the short-term. But the long-term response of seizures and neuropsychological regression has not been systematically studied. Clobazam is a benzodiazepine derivative with antiepileptic properties. The pharmacologic properties of clobazam make of this drug a particularly useful option in ESES: in patients with ESES the alpha-2 subunit of the GABA receptor is preferentially up-regulated and clobazam may have a higher affinity for this particular subunit, so investigators expect a higher effect of this drug on ESES patients than with other benzodiazepines (Loddenkemper et al, in preparation). The aim of our study is to objectively evaluate the response to clobazam treatment of neuropsychological deficits, seizures and abnormal discharges in the EEG in patients with ESES. Clobazam treatment is used in routine clinical practice and investigators will objectively quantify its effect. Our working hypothesis is that high-dose clobazam treatment may control the abnormal epileptiform discharges in the EEG in patients with ESES. The reduction in abnormal epileptiform discharges may lead to an improvement in neuropsychological deficits and seizures. The predicted improvement in seizures and neuropsychological function would lead to a better quality of life in these patients.

Who is eligible to participate?

Inclusion Criteria: - A change in treatment regimen is required because of very active epileptiform activity during sleep and neuropsychological regression. This means that only patients with very active epileptiform activity and, therefore, with a need for a change in treatment regimen as clinically indicated will be potentially eligible. Additionally, a prior clinical decision to use clobazam should have been made by the primary epileptologist in order to participate in the study. - Availability for clinical, neuropsychological and EEG follow-up. Exclusion Criteria: - Electro-clinical evolution that does not require a change in medication regimen or changes in medication regimen that do not include clobazam.

Which medical condition, disease, disorder, syndrome, illness, or injury is researched?

Electrical Status Epilepticus in Sleep

Study Interventions

Interventions can include giving participants drugs, medical devices, procedures, vaccines, and other products that are either investigational or already available or noninvasive approaches such as surveys, education, and interviews.

Drug:ESES treated with clobazamThe patients that will participate in the protocol will be those that are administered for clinical reasons oral clobazam.

Study Arms

Research studies and clinical trials typically have two or more research arms. An arm is a group of people who receive the same treatment in the study.

ESES treated with clobazamThe patients that will participate in the protocol will be those that are administered for clinical reasons oral clobazam.

Study Status

Completed

Start Date: July 2012

Completed Date: July 2015

Phase: N/A

Type: Observational [Patient Registry]

Design:

Primary Outcome: Cognitive ability

Secondary Outcome: Interictal epileptiform activity

Study sponsors, principal investigator, and references

Principal Investigator: Tobias Loddenkemper, MD

Lead Sponsor: Boston Children’s Hospital

Collaborator: Lundbeck LLC

More information:https://clinicaltrials.gov/show/NCT02127918

Fernández IS, Chapman KE, Peters JM, Kothare SV, Nordli DR Jr, Jensen FE, Berg AT, Loddenkemper T. The tower of Babel: survey on concepts and terminology in electrical status epilepticus in sleep and continuous spikes and waves during sleep in North America. Epilepsia. 2013 Apr;54(4):741-50. doi: 10.1111/epi.12039. Epub 2012 Nov 16.

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