Work

Successful application of vagus nerve stimulation in super refractory status epilepticus associated with MERRF syndrome

Year: 2025

Type: article

Abstract: • VNS implantation during SRSE can stop seizures and prevent recurrence effectively. • VNS may be a promising therapy for SRSE in mitochondrial epilepsy syndromes. • Rapid VNS titration may enhance outcomes by accelerating efficacy without major side effects. 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.

Source: Epilepsy & Behavior Reports

DOI: 10.1016/j.ebr.2025.100769

Authors: Leyla Baysal, Sabrina Jobi, Simone Zimmermann, Ann-Kristin Helmers, Nils Gerd Margraf

Institution: University Hospital Schleswig-Holstein

Language: English

Topic: Vagus Nerve Stimulation Research

Subfield: Neurology

Field: Neuroscience

Domain: Life Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $2,630