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Epilepsy Behav Case Rep. 2014 Sep 29;2:164-6. doi: 10.1016/j.ebcr.2014.09.003. eCollection 2014.

Seizure control and improvement of neurological dysfunction in Lafora disease with perampanel.

Epilepsy & behavior case reports

Maya Dirani, Wassim Nasreddine, Fatima Abdulla, Ahmad Beydoun

Affiliations

  1. Division of Pediatric Neurology, American University of Beirut Medical Center, Lebanon.
  2. Department of Neurology, American University of Beirut Medical Center, Lebanon.
  3. Department of Clinical Neurosciences, Salmaniya Medical Complex, Bahrain.

PMID: 25667898 PMCID: PMC4307869 DOI: 10.1016/j.ebcr.2014.09.003

Abstract

Lafora disease is a rare and fatal disease characterized by seizures, progressive cognitive and behavioral deterioration, as well as cerebellar dysfunction. Currently, there is no efficacious treatment that will control the seizures and improve the cognitive decline in this disease. We report a patient with Lafora disease who experienced a dramatic amelioration in her seizure frequency as well as the associated neurological and cognitive dysfunction following initiation of treatment with perampanel administered as monotherapy. Perampanel is the first potentially efficacious treatment for Lafora disease. We discuss a potential mechanism for the efficacy of perampanel in this disease.

Keywords: Anticonvulsants; Lafora disease; Perampanel; Progressive myoclonic epilepsy

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