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Malignant autosomal dominant frontal lobe epilepsy with repeated episodes of status epilepticus: successful treatment with vagal nerve stimulation


Epileptic Disorders. Volume 12, Number 2, 155-8, June 2010, Clinical commentary with video sequences

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Author(s) : Mar Carreño, Dionisio Garcia-Alvarez, Iratxe Maestro, Santiago Fernández, Antonio Donaire, Teresa Boget, Jordi Rumià, Luis Pintor, Xavier Setoain

Summary : Autosomal dominant nocturnal frontal lobe epilepsy (ADNFLE) is a familial partial epilepsy syndrome characterized by seizures suggesting a frontal lobe origin occurring predominantly during sleep. Up to a third of patients may have refractory seizures, with repeated episodes of status epilepticus, intellectual disability of variable degree and psychiatric disturbances. We report a patient with ADNFLE, refractory seizures and repeated episodes of life-threatening convulsive status epilepticus who underwent prolonged video-EEG monitoring and was implanted with a vagal nerve stimulator. At 3.5 years of follow-up, a decrease of more than 80% in seizure frequency was achieved, episodes of status were completely controlled and he displayed improved mood and alertness. Vagal nerve stimulation may be considered as therapy for patients with refractory epilepsies of genetic cause, as well as repeated status epilepticus.

Keywords : epilepsy, nocturnal frontal lobe epilepsy, ADNFLE, vagal nerve stimulator, familial, VNS

 

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