Cardiac rhythm in Dravet Syndrome: cause of SUDEP?

  • Research type

    Research Study

  • Full title

    Pilot study of cardiac rhythm in Dravet Syndrome: cause of SUDEP?

  • IRAS ID

    130540

  • Contact name

    Sanjay Sisodiya

  • Contact email

    s.sisodiya@ucl.ac.uk

  • Sponsor organisation

    UCL

  • Research summary

    Dravet Syndrome is an early onset epilepsy characterised by multiple seizure types, including recurrent status epilepticus, often aggravated by fever and/or illness. Children have a poor prognosis for both seizure control and neurodevelopmental outcome. They are also at higher risk for sudden unexpected death in epilepsy. Most people, adults and children, with Dravet Syndrome have abnormalities in one particular sodium channel gene. Sodium channels may be found in other organs of the body, especially the heart, and abnormalities may be found in individuals prone to disruption of heart rhythm. We propose that children with Dravet Syndrome also have abnormalities in sodium channels in the heart, and plan to look at heart rhythm in people with Dravet Syndrome both when well and at the time of illness.

    We plan a pilot study evaluating ECG, both routine and 24 hour recordings, on 20 children with Dravet Syndrome both when they are well and also at times when they are unwell. This study will determine whether cardiac abnormalities may be aggravated at times of illness, which in turn would have implications for management and monitoring at times of illness. Identification of illness-related ECG abnormalities might also lead to studies of acute preventative therapies at such times.

  • REC name

    London - Queen Square Research Ethics Committee

  • REC reference

    14/LO/0033

  • Date of REC Opinion

    11 Mar 2014

  • REC opinion

    Further Information Favourable Opinion