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Cardiac rhythm in Dravet Syndrome: a cause of SUDEP?

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.  Previous studies have evaluated cardiac parameters in a small number of children with Dravet Syndrome and controls, utilising routine and 24 hour ECG.

Neither showed any abnormality in quantitative parameters, but they did both show significantly lower values for heart rate variability in children with Dravet Syndrome as compared to  controls, suggesting autonomic dysfunction.


To determine whether heart rhythm abnormalities are aggravated at times of illness in children with Dravet Syndrome and establish whether this in turn has implications for management and monitoring at times of illness. We hope that this  might lead to studies of acute preventative therapies at such times.

What this means

We know that people with Dravet Syndrome are susceptive to additional heart problems. We want to know if heart problems can arise from periods of illness such as having a cold or a fever. If so, then we will work to change guidelines for support of patients with Dravet Syndrome in times of illness and ensure heart problems are considered.


The study involves parents retaining an ECG machine at home, and recording the heart rhythm during illness. Eight families have been recruited and have participated to date. Data collection is ongoing but slow as this is a rare condition.


Helen Cross, Juan Pablo Kaski, Sarah Aylett, Elaine Hughes, Sanjay Sisodiya, Katharina Vezyroglou

Cyber EssentialsFundraising RegulatorYoung Epilepsy is the operating name of The National Centre for Young People with Epilepsy (NCYPE).
Registered Charity number 311877 (England and Wales)

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