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Updated: May 10, 2025

Vagus Nerve Stimulation As an Adjunctive Neurostimulation Tool in Treatment-resistant Depression
Published on: January 7, 2019
Successful application of vagus nerve stimulation in super refractory status epilepticus associated with MERRF
Leyla Baysal1, Sabrina Jobi1, Simone Zimmermann1
1Epilepsy Center, Department of Neurology, University Hospital Schleswig-Holstein, Campus Kiel, Arnold-Heller-Str. 3, 24105 Kiel, Germany.
Abstract:
Myoclonic epilepsy with ragged red fibers (MERRF) syndrome is a rare mitochondrial disorder marked by myoclonus, ataxia, cognitive impairment, myopathy, and sensorineural hearing loss. Seizures in MERRF syndrome are often resistant to standard antiseizure medication. We present a unique case of an adult patient with MERRF syndrome, caused by the m.8344A > G point mutation, who developed super-refractory status epilepticus (SRSE), successfully managed by the initiation and rapid escalation of vagus nerve stimulation (VNS) therapy. Over a two-year follow-up period, the VNS therapy enabled a long-term control of generalized tonic-clonic seizures. A further status epilepticus has not yet occurred. VNS may therefore be a promising therapeutic option for managing SRSE in patients with mitochondrial epilepsy syndromes.

