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Cardiac Arrhythmias in Dravet Syndrome

The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our disclaimer for details.
 
ClinicalTrials.gov Identifier: NCT02415686
Recruitment Status : Completed
First Posted : April 14, 2015
Last Update Posted : September 7, 2018
Sponsor:
Collaborator:
Epilepsiefonds
Information provided by (Responsible Party):
Stichting Epilepsie Instellingen Nederland

Brief Summary:

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.


Condition or disease
Epilepsy

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Study Type : Observational
Actual Enrollment : 59 participants
Observational Model: Case-Control
Time Perspective: Prospective
Official Title: Cardiac Arrhythmias in Dravet Syndrome: an Observational, International, Multicentre Study
Actual Study Start Date : June 2015
Actual Primary Completion Date : August 29, 2018
Actual Study Completion Date : August 29, 2018





Primary Outcome Measures :
  1. 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 ]

Secondary Outcome Measures :
  1. 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 ]


Information from the National Library of Medicine

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Ages Eligible for Study:   6 Years and older   (Child, Adult, Older Adult)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   No
Sampling Method:   Non-Probability Sample
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.

Criteria

Criteria:

Cases must meet all of the following criteria:

  1. DS with a known pathogenic SCN1A mutation
  2. seizure frequency ≥ 1/week (all seizure types expect for absences or myoclonias)
  3. no self-harm
  4. age ≥ 6 years

Each case will be matched to two historical controls (age +/- 5 years). Controls will meet the following criteria:

  1. definite diagnosis of epilepsy
  2. no clinical suspicion of DS
  3. at least two seizures recorded (all seizure types expect for absences or myoclonias) during video-EEG registration.
  4. age ≥ 6 years

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT02415686


Locations
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Germany
Universität Bonn
Bonn, North Rhine-Westphalia, Germany, 53113
Netherlands
Stichting Epilepsie Instellingen Nederland (SEIN)
Heemstede, Achterweg 5, Netherlands, 2103 SW
United Kingdom
Great Ormond Street Hospital
London, South East, United Kingdom, WC1N 3JH
Sponsors and Collaborators
Stichting Epilepsie Instellingen Nederland
Epilepsiefonds
Investigators
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Principal Investigator: Roland Thijs, Dr. Stichting Epilepsie Instellingen Nederland (S.E.I.N.)
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Responsible Party: Stichting Epilepsie Instellingen Nederland
ClinicalTrials.gov Identifier: NCT02415686    
Other Study ID Numbers: NL48765.058.15
First Posted: April 14, 2015    Key Record Dates
Last Update Posted: September 7, 2018
Last Verified: November 2017
Keywords provided by Stichting Epilepsie Instellingen Nederland:
Dravet syndrome
Sudden Unexpected Death in Epilepsy (SUDEP)
Arrhythmias
Additional relevant MeSH terms:
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Epilepsy
Epilepsies, Myoclonic
Arrhythmias, Cardiac
Pathologic Processes
Brain Diseases
Central Nervous System Diseases
Nervous System Diseases
Heart Diseases
Cardiovascular Diseases
Epilepsy, Generalized
Epileptic Syndromes