- ICH GCP
- US Clinical Trials Registry
- Clinical Trial NCT02415686
Cardiac Arrhythmias in Dravet Syndrome
Cardiac Arrhythmias in Dravet Syndrome: an Observational, International, Multicentre Study
SUMMARY
Rationale:
People with Dravet Syndrome (DS), a rare epilepsy syndrome, have a high risk of Sudden Unexpected Death in Epilepsy (SUDEP). Mouse models indicated that the responsible sodium channel mutation (SCN1A) not only alters cortical excitability but also increases the propensity to arrhythmias. Little is known yet about the prevalence of seizure-induced arrhythmias in human DS subjects.
Objective:
To assess the prevalence of cardiac arrhythmias in DS and to compare the prevalence of cardiac arrhythmias between DS subjects and subjects with other types of epilepsy.
Study design:
Observational study.
Study population:
Subjects with Dravet syndrome and a known pathogenic SCN1A mutation, seizure frequency ≥ 1/week (all seizure types except for absences or myoclonias), age ≥ 6 years and no signs of self-harm. Each case will be matched to two historical controls (age +/- 5 years) from the EEG databases of the participating centres. Only those controls with two or more recorded seizures will be matched to the cases.
Intervention:
Not applicable
Main study parameters/endpoints:
Ictal asystole Ictal bradycardia Ictal QT-shortening/lengthening
Nature and extent of the burden and risks associated with participation, benefit and group relatedness:
Participation does not carry risks. The sensor is wearable and miniaturised, thus minimising discomfort. If this nevertheless may occur, the study can be terminated. This study provides specific tools to investigate the seizure-related heart rate response. Subjects may thus benefit from participation by identification of otherwise unknown arrhythmias. The rationale of the study (the high SUDEP risk and the evidence in animal studies for arrhythmic cause of sudden death) specifically applies to DS, a rare epileptic syndrome including minors and incapacitated persons. The investigators believe that the lack of risks, the potential diagnostic benefit, the minimal intervention with novel and wearable sensors and the possibility to terminate the study in case of discomfort, justifies the study in this patient group.
Study Overview
Status
Conditions
Study Type
Enrollment (Actual)
Contacts and Locations
Study Locations
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North Rhine-Westphalia
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Bonn, North Rhine-Westphalia, Germany, 53113
- Universitat Bonn
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Achterweg 5
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Heemstede, Achterweg 5, Netherlands, 2103 SW
- Stichting Epilepsie Instellingen Nederland (SEIN)
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South East
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London, South East, United Kingdom, WC1N 3JH
- Great Ormond Street Hospital
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Participation Criteria
Eligibility Criteria
Ages Eligible for Study
Accepts Healthy Volunteers
Genders Eligible for Study
Sampling Method
Study Population
the Netherlands (coordinating centre: SEIN). Procedure: The UMCU Medical Genetics department is the key referral centre in the Netherlands for DS (Brilstra, UMCU). Cases will be recruited from the UMCU database. Prior to inclusion, a panel (Gunning, Brilstra, Thijs) will review clinical data to ensure diagnostic consistency.
Additional DS subjects will be recruited from the local DS databases at Universität Bonn (25 subjects; 4 adults; local coordinator R Surges) and UCL (100 subjects; 15 adults; local coordinator S Sisodiya).
We will select historical controls (subjects with epilepsy without DS) from the video-EEG databases of the participating centres.
Description
Criteria:
Cases must meet all of the following criteria:
- DS with a known pathogenic SCN1A mutation
- seizure frequency ≥ 1/week (all seizure types expect for absences or myoclonias)
- no self-harm
- age ≥ 6 years
Each case will be matched to two historical controls (age +/- 5 years). Controls will meet the following criteria:
- definite diagnosis of epilepsy
- no clinical suspicion of DS
- at least two seizures recorded (all seizure types expect for absences or myoclonias) during video-EEG registration.
- age ≥ 6 years
Study Plan
How is the study designed?
Design Details
What is the study measuring?
Primary Outcome Measures
Outcome Measure |
Time Frame |
---|---|
Ictal asystole (sinus arrest ≥ 3 s) or ictal bradycardia (< 2nd heart rate percentile for age)
Time Frame: We will record heart rate patterns during seizures with miniaturized wearable EKG-monitors for 2 periods of 10 days
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We will record heart rate patterns during seizures with miniaturized wearable EKG-monitors for 2 periods of 10 days
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Secondary Outcome Measures
Outcome Measure |
Time Frame |
---|---|
Ictal QT lengthening or shortening
Time Frame: We will record heart rate patterns during seizures with miniaturized wearable EKG-monitors for 2 periods of 10 days
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We will record heart rate patterns during seizures with miniaturized wearable EKG-monitors for 2 periods of 10 days
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Collaborators and Investigators
Collaborators
Investigators
- Principal Investigator: Roland Thijs, Dr., Stichting Epilepsie Instellingen Nederland (S.E.I.N.)
Study record dates
Study Major Dates
Study Start (ACTUAL)
Primary Completion (ACTUAL)
Study Completion (ACTUAL)
Study Registration Dates
First Submitted
First Submitted That Met QC Criteria
First Posted (ESTIMATE)
Study Record Updates
Last Update Posted (ACTUAL)
Last Update Submitted That Met QC Criteria
Last Verified
More Information
Terms related to this study
Additional Relevant MeSH Terms
Other Study ID Numbers
- NL48765.058.15
This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.
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