title: Neuropsychological findings in patients with Unverricht-Lundborg disease.
abstract: The aims of this study were to clarify if patients with Unverricht-Lundborg disease (ULD) have adequate cognitive functioning and to delineate their neuropsychological profile. We evaluated 20 patients with ULD and 20 healthy, matched controls. Mean age of the patients was 35 years, and mean duration of disease, 22 years. Patients underwent a neuropsychological battery exploring intelligence, executive functions, visuospatial and verbal memory, depression, and anxiety. Eleven of 20 subjects with ULD had mild to moderate cognitive impairment. Compared with controls, patients with ULD had lower scores on all short-term memory and executive function tasks. Linear regression analysis disclosed significant associations between impaired performance on some memory tests and duration of disease and between severity of myoclonus and performance on most executive function tests. In conclusion, most patients with ULD seem to be impaired with respect to cognitive abilities. Longitudinal prospective studies are needed to confirm and further expand our findings.
authors.affiliation: IRCCS Centro Neurolesi Bonino Pulejo, Messina, Italy. edoferl@hotmail.it
authors.forename: Edoardo
authors.initials: E
authors.lastname: Ferlazzo
authors.forename: Antonella
authors.initials: A
authors.lastname: Gagliano
authors.forename: Tiziana
authors.initials: T
authors.lastname: Calarese
authors.forename: Adriana
authors.initials: A
authors.lastname: Magaudda
authors.forename: Pasquale
authors.initials: P
authors.lastname: Striano
authors.forename: Lara
authors.initials: L
authors.lastname: Cortese
authors.forename: Clemente
authors.initials: C
authors.lastname: Cedro
authors.forename: Virgilie
authors.initials: V
authors.lastname: Laguitton
authors.forename: Placido
authors.initials: P
authors.lastname: Bramanti
authors.forename: Marilena
authors.initials: M
authors.lastname: Carbonaro
authors.forename: Addolorata
authors.initials: A
authors.lastname: Albachiara
authors.forename: Nina
authors.initials: N
authors.lastname: Fragassi
authors.forename: Domenico
authors.initials: D
authors.lastname: Italiano
authors.forename: Edoardo
authors.initials: E
authors.lastname: Sessa
authors.forename: Antonietta
authors.initials: A
authors.lastname: Coppola
authors.forename: Pierre
authors.initials: P
authors.lastname: Genton
mesh_terms: D000293:Adolescent; D000328:Adult; D001007:Anxiety / Q000150:complications / Q000523:psychology; D003071:Cognition / Q000502:physiology; D003863:Depression / Q000150:complications / Q000523:psychology; D005260:Female; D006801:Humans; D007361:Intelligence Tests; D016014:Linear Models; D008137:Longitudinal Studies; D008297:Male; D008568:Memory / Q000502:physiology; D008875:Middle Aged; D009207:Myoclonus / Q000209:etiology / Q000523:psychology; D009483:Neuropsychological Tests; D011597:Psychomotor Performance / Q000502:physiology; D020194:Unverricht-Lundborg Syndrome / Q000523:psychology*; D014796:Visual Perception / Q000502:physiology; D055815:Young Adult