Perampanel attenuates myoclonus in a patient with neuronal ceroid lipofuscinoses type 2 disease
Neuronal ceroid lipofuscinoses type 2 disease (CLN2) is a very rare, autosomal recessive neurodegerative disease caused by deficient activity of the enzyme tripeptidyl peptidase 1 (TPP1). The seizures in CLN2 are polymorphic and resistant to antiepileptic drugs. In particular, myoclonus (epileptic and non-epileptic) predominant as the disease progresses. Herein, we present a child of CLN2 disease, who had near-continuous myoclonus, and was subsequently attenuated by administration of Perampanel.
Source: Brain and Development - Category: Neurology Authors: Lee Chin Wong, Chia-Jui Hsu, Wang-Tso Lee Source Type: research