DOAJ Open Access 2025

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

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

Abstrak

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.

Topik & Kata Kunci

Neurology. Diseases of the nervous system Neurophysiology and neuropsychology

Penulis (5)

Leyla Baysal

Sabrina Jobi

Simone Zimmermann

Ann-Kristin Helmers

Nils Gerd Margraf

Format Sitasi

APA MLA BibTeX

Baysal, L., Jobi, S., Zimmermann, S., Helmers, A., Margraf, N.G. (2025). Successful application of vagus nerve stimulation in super refractory status epilepticus associated with MERRF syndrome. https://doi.org/10.1016/j.ebr.2025.100769

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Informasi Jurnal

Tahun Terbit: 2025
Sumber Database: DOAJ
DOI: 10.1016/j.ebr.2025.100769
Akses: Open Access ✓