title: Cognitive and neuroradiological findings in myotonic dystrophy.
abstract: Cognitive functions were investigated in 37 patients with myotonic dystrophy (MD) and correlated with clinical and neuroradiological variables. The whole cognitive performance was at a low-average level; in about 1/3 of the subjects, in fact, the scores at the neuropsychological tests were below the normal range. There was a consistent trend for patients with inheritance on maternal side to perform worse on Wechsler verbal score and to present cerebral atrophy. In 7 out of 12 subjects focal white matter lesions were found at nuclear magnetic resonance. The significance of these findings and its relation to cognitive performance are discussed.
authors.affiliation: 3rd Department of Neurology, University of Pavia, Italy.
authors.forename: E
authors.initials: E
authors.lastname: Sinforiani
authors.forename: G
authors.initials: G
authors.lastname: Sandrini
authors.forename: A
authors.initials: A
authors.lastname: Martelli
authors.forename: M
authors.initials: M
authors.lastname: Mauri
authors.forename: C
authors.initials: C
authors.lastname: Uggetti
authors.forename: G
authors.initials: G
authors.lastname: Bono
authors.forename: G
authors.initials: G
authors.lastname: Nappi
mesh_terms: D000328:Adult; D000368:Aged; D001284:Atrophy; D001921:Brain / Q000473:pathology; D002552:Cerebral Ventricles / Q000473:pathology; D005260:Female; D005799:Genes, Dominant / Q000235:genetics; D006801:Humans; D007360:Intelligence / Q000235:genetics; D008137:Longitudinal Studies; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009223:Myotonic Dystrophy / Q000175:diagnosis* / Q000235:genetics; D009460:Neurologic Examination; D009483:Neuropsychological Tests; D014057:Tomography, X-Ray Computed