Efficacy and tolerability of perampanel in ten patients with Lafora disease

Danielle Goldsmith, Berge A. Minassian

Research output: Contribution to journalArticle

33 Citations (Scopus)

Abstract

Lafora disease (LD) is a fatal intractable adolescence-onset progressive myoclonus epilepsy. Recently, two single-case studies reported drastic reductions in seizures and myoclonus with the AMPA antagonist perampanel and improved activities of daily living. We proceeded to study the effect of perampanel on 10 patients with genetically confirmed LD with disease duration ranging from 2 to 27 years. Open-label perampanel was added to ongoing medications to a mean dose of 6.7 mg/day. Seizures, myoclonus, functional disability, and cognition scores were measured for the third and ninth months following initiation and compared with those of the month prior to the start of therapy. Three patients withdrew because of inefficacy or side effects. Four had significant reduction in seizures of greater than 74% from baseline. Seven had major improvement in myoclonus with group-adjusted sum score of myoclonus intensity reduced from 7.01 at baseline to 5.67 and 5.18 at 3 and 9 months, respectively. There was no significant improvement in disability and cognition. While not universal, perampanel adjunctive therapy appears to confer particular benefit not commonly seen with other antiepileptic drugs on seizures and myoclonus in LD. Improvement in the extremely disabling myoclonus of LD is a major benefit in this devastating disease.

Original languageEnglish (US)
Pages (from-to)132-135
Number of pages4
JournalEpilepsy and Behavior
Volume62
DOIs
StatePublished - Sep 1 2016

Fingerprint

Lafora Disease
Myoclonus
Seizures
Cognition
Progressive Myoclonic Epilepsy
alpha-Amino-3-hydroxy-5-methyl-4-isoxazolepropionic Acid
Activities of Daily Living
Anticonvulsants
perampanel
Therapeutics

Keywords

  • Antiepileptic
  • Lafora disease
  • Perampanel
  • Progressive myoclonus epilepsy

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology
  • Behavioral Neuroscience

Cite this

Efficacy and tolerability of perampanel in ten patients with Lafora disease. / Goldsmith, Danielle; Minassian, Berge A.

In: Epilepsy and Behavior, Vol. 62, 01.09.2016, p. 132-135.

Research output: Contribution to journalArticle

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