Naslov
Vagus nerve stimulation in a patient with progressive myoclonus epilepsy: a second reported case in the world

Autori
Hajnšek, Sanja ; Petelin Gadže, Željka ; Nanković, Sibila ; Mrak, Goran ; Borovečki, Fran ; Šulentić, Vlatko ; Kovačević, Ivana ; Bujan Kovač, Andreja

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, stručni

Izvornik
Epilepsia, Vol. 54 (suppl.3) / - , 2013, 183-183

Skup
30th International Epilepsy Congress

Mjesto i datum
Montréal, Kanada, 23–27.06.2013

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
Vagus nerve stimulation, progressive myoclonus epilepsy

Sažetak
Background: Progressive myoclonus epilepsies (PME) are rare inherited neurodegenerative diseases with great clinical and genetic differences, as well as poor prognosis. The syndrome includes myoclonic seizures and generalized tonic-clonic seizures (GTCS) that are often drug resistant, as well as progressive neurological decline involving cerebellar, pyramidal, extrapyramidal symptoms and dementia. So, vagus nerve stimulation (VNS) is a standard treatment for drug resistant epilepsy, it is surprising that we found only one single case report of VNS implantation in PME-Unverricht- Lundborg disease, 12 years ago. Case Presentation: We report another case of the utility of VNS for PME in a 19-year-old Caucasian male patient. The clinical diagnosis of our patient is probably Lafory body disease. The patient had progressive myoclonus, GTCS, cerebellar and extrapyramidal symptomatology, as well as dementia. After VNS implantation, in the 10-month follow-up period, patient's clinical condition and quality of life improved. There was significant regression of myoclonus, moderate regression of cerebellar symptomatology, and following VNS implantation patient did not experience any more GTCS. Conclusion: VNS therapy may be considered a treatment option for progressive myoclonus epilepsy. Further clinical studies are needed to confirm the clinical effects produced by chronic vagus stimulation in patients with progressive myoclonus epilepsies.

Izvorni jezik
Hrvatski

Znanstvena područja
Kliničke medicinske znanosti

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