Dravet syndrome and deep brain stimulation: seizure control after 10 years of treatment

Dravet syndrome is a genetically determined severe epilepsy associated with cognitive decline and ataxia. The many types of seizures seen in these patients are typically pharmacoresistant. Here we describe two adults with Dravet syndrome who were treated with thalamic deep brain stimulation (DBS) and followed for 10 years. One patient with partial onset seizures received DBS at age 19 and showed a marked improvement in seizure control after DBS insertion and stimulation. The other patient with generalized onset seizures received DBS at age 34 and did not show any immediate benefit. No side effects or changes in cognition were observed in either of the patients. This is the first report of (short- and) long-term results in Dravet patients treated with thalamic DBS. We speculate that the results of DBS for epilepsy in patients with Dravet syndrome may be related to age at initiation of DBS treatment and seizure type.