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. 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

Maya Dirani  1 , Wassim Nasreddine  2 , Fatima Abdulla  3 , Ahmad Beydoun  2
Affiliations

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

Maya Dirani et al. Epilepsy Behav Case Rep. .

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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