Please use this identifier to cite or link to this item: https://biore.bio.bg.ac.rs/handle/123456789/3922
Title: Is adjunctive perampanel beneficial for lafora disease?
Authors: Stevanovic, Galina
Jovic, Nebojsa
Kecmanović, Miljana 
Keywords: lafora disease;diagnosis;anticolvusants;perampanel;treatment outcome
Issue Date: 2020
Rank: M23
Journal: Vojnosanitetski pregled
Series/Report no.: 77 (5);539-544
Abstract: 
Backgrund/Aim. Lafora disease (LD) is progressive myoclonus epilepsy, characterized by intractable myoclonus and
seizures, inevitable neurological deterioration, brutal cognitive decline and poor prognosis. The treatment still remains
purely symptomatic. Recently, two single-case studies and
one case series study reported the favourable effects of perampanel in LD. Our study aimed to test the benefits reported in three separate case studies. Methods. We performed an open label, prospective study of 4 patients aged
between 22 and 34 years with mutation in NHLRC1
(EPM2B) gene, treated with perampanel (6–8 mg/day) as
add-on therapy. Follow-up period comprised 14–26
months. Seizure frequency, myoclonus, functional disability
and cognitive performance were analysed. Results. In 3 patients, both, seizures and myoclonus, showed remarkable
improvement after the drug introduction (> 50% reduction). No significant effect was seen in one case. The functional and cognitive impairment maintained at the same level, though all patients were at the later stage of the disease.
Psychiatric side effects were dose related. Conclusion. Our
study supports the rare, previously reported observations
that perampanel is beneficial in treating LD patients.
URI: https://biore.bio.bg.ac.rs/handle/123456789/3922
ISSN: 0042-8450
2406-0720
DOI: 10.2298/VSP170416013S
Appears in Collections:Journal Article

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