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

## TL;DR

Vagus nerve stimulation (VNS) successfully controlled seizures in a rare mitochondrial disorder called MERRF syndrome, offering a new treatment option for drug-resistant epilepsy.

## Contribution

This case demonstrates VNS as a promising therapy for super refractory status epilepticus in mitochondrial epilepsy syndromes.

## Key findings

- VNS implantation during SRSE effectively stopped seizures and prevented recurrence.
- Rapid VNS titration accelerated efficacy without major side effects.
- Two-year follow-up showed long-term control of generalized tonic-clonic seizures.

## 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.

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.