Neuropediatrics 2006; 37 - P64
DOI: 10.1055/s-2006-974075

Sodium channel influencing drugs may provoke refractory status epilepticus in patients with SCN1A-mutations!

GJ Kluger 1, B Neubauer 2, K Reiter 3, A Zsoter 1, F Heinen 3, T Nikolai 3, H Holthausen 1
  • 1Klinik für Neuropädiatrie und Neurologische Rehabilitation, Epilepsiezentrum für Kinder und Jugendliche, BHZ Vogtareuth, Vogtareuth, Germany
  • 2Universitätskinderklinik Gießen, Neuropädiatrie, Gießen, Germany
  • 3Dr. von Haunersches Kinderspital, München, Germany

Aims: Sodium channels play an important role in neuronal excitability of brain, heart and muscle tissue. In patients with SCN1A mutations (e.g. severe myoclonic epilepsy of infancy/Dravet syndrome; infantile epilepsy with grand-mal seizures/“frühkindliche Grand-mal-Epilepsie“) drugs as PHT, CBZ, OXC and LTG often aggravate seizures due to their mode of action action via sodium-channels.

We report 2 children with SCN1A-mutations and unusual refractory status epilepticus after febrile seizures resulting in severe brain damage and discuss the potential influence of sodium channel influencing drugs.

Patients: 2 healthy children (one boy and one girl) developed at the ages of 28 and 13 months respectively long lasting status epilepticus following viral infections, which led to long term ventilation, cardiovascular failure and cerebral oedema, and as a result of this severe sequela. In both patients SCN1A mutations (truncated mutations, Arg 1235x and Arg535fsX540) were detected later on. Drug therapy in both children consisted of intravenous phenytoin, clonidin, propofol, thiopental and catecholamine, all with a mode of action on sodium channels.

Discussion: Different drugs (antiepileptic drugs, muscle relaxants, narcotic drugs, antiarrythmic drugs and catecholamines) have a mode of action on sodium channels. Some of them have been intravenously administered in both of the reported patients in different combinations. This might be an explanation for the exceptionally severe course of the status epilepticus and the difficult intensive care treatment.

Conclusion: In children with focal and/or long lasting febrile seizures – especially in the first two years of life – SCN1A mutations should be taken into consideration because of the fact that sodium channel influencing drugs might aggravate seizures and also lead to further cardiac and circulatory depression. This is also true for older patients with difficult to treat epilepsies of unknown etiology and a history of febrile seizures.