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

Abstract

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.

• Authors: Mar Carreño, Dionisio Garcia-Alvarez, Iratxe Maestro, Santiago Fernández, Antonio Donaire, Teresa Boget, Jordi Rumià, Luis Pintor, Xavier Setoain
• Journal: Epileptic disorders : international epilepsy journal with videotape (Epileptic Disord) Vol. 12 Issue 2 Pg. 155-8 (Jun 2010) ISSN: 1294-9361 [Print] France
• PMID: 20478764 (Publication Type: Case Reports, Journal Article)

Topics

• Adult
• Chromosome Aberrations
• Electroencephalography
• Epilepsy, Frontal Lobe (genetics, therapy)
• Follow-Up Studies
• Humans
• Male
• Status Epilepticus (genetics, therapy)
• Treatment Outcome
• Vagus Nerve Stimulation
• Video Recording