title: A comprehensive neuropsychological description of cognition in drug-refractory juvenile myoclonic epilepsy.
abstract: The study of juvenile myoclonic epilepsy is important in that: it is common and heterogeneous; the etiology is unknown; and patients report broad cognitive problems. We utilized a broad battery of neuropsychometric tests to assess the following: intellectual function, memory, language and naming, executive function, the impact of epilepsy, and antiepilepsy drug side effects. Sixty people with drug-refractory JME were interviewed, and performance was profoundly impaired across the range of tests. Impairments included the following: full-scale IQ (89, p<0.001); processing speed (86, p<0.001); visual memory (immediate and delayed) more affected than verbal memory; verbal fluency and inhibition (p<0.001); and self-reported drug side effects (p<0.001). Eighty-three percent of patients exhibited frank executive dysfunction, which was moderate to severe in 66%. Regression modeling confirmed that an early age at onset and the need for polytherapy were associated with poorer cognitive outcomes. This study confirms previous reports of executive dysfunction in a larger cohort and with greater statistical rigor. We also identified a high prevalence of neurotoxicity symptoms such as fatigue and poorer functioning across intellectual and memory tests than had previously been reported.
authors.affiliation: Wales Epilepsy Research Network (WERN), College of Medicine, Swansea University, Swansea SA2 8PP, UK. Electronic address: Rhys-Thomas@doctors.org.uk.
authors.forename: Rhys H
authors.initials: RH
authors.lastname: Thomas
authors.affiliation: Department of Clinical and Molecular Pharmacology, University of Liverpool, Liverpool, UK. Electronic address: jordana.walsh@googlemail.com.
authors.forename: Jordana
authors.initials: J
authors.lastname: Walsh
authors.affiliation: Wales Epilepsy Research Network (WERN), College of Medicine, Swansea University, Swansea SA2 8PP, UK. Electronic address: carla.church@hotmail.co.uk.
authors.forename: Carla
authors.initials: C
authors.lastname: Church
authors.affiliation: Department of Clinical and Molecular Pharmacology, University of Liverpool, Liverpool, UK.
authors.forename: Graeme J
authors.initials: GJ
authors.lastname: Sills
authors.affiliation: Department of Clinical and Molecular Pharmacology, University of Liverpool, Liverpool, UK. Electronic address: A.G.Marson@liverpool.ac.uk.
authors.forename: Anthony G
authors.initials: AG
authors.lastname: Marson
authors.affiliation: Department of Clinical and Molecular Pharmacology, University of Liverpool, Liverpool, UK. Electronic address: G.Baker@liverpool.ac.uk.
authors.forename: Gus A
authors.initials: GA
authors.lastname: Baker
authors.affiliation: Wales Epilepsy Research Network (WERN), College of Medicine, Swansea University, Swansea SA2 8PP, UK. Electronic address: M.I.Rees@swansea.ac.uk.
authors.forename: Mark I
authors.initials: MI
authors.lastname: Rees
mesh_terms: D000927:Anticonvulsants / Q000627:therapeutic use; D001288:Attention; D003072:Cognition Disorders / Q000175:diagnosis* / Q000209:etiology*; D015331:Cohort Studies; D056344:Executive Function; D005260:Female; D006801:Humans; D007360:Intelligence; D008279:Magnetic Resonance Imaging; D008297:Male; D008569:Memory Disorders / Q000175:diagnosis / Q000209:etiology; D020190:Myoclonic Epilepsy, Juvenile / Q000150:complications* / Q000188:drug therapy; D009483:Neuropsychological Tests; D012044:Regression Analysis; D011795:Surveys and Questionnaires; D014706:Verbal Learning
chemical_list: D000927:Anticonvulsants
keywords: Cognition; Executive function; Juvenile myoclonic epilepsy; Memory; Psychology