title: Cortical atrophy and cognitive performance in infantile nephropathic cystinosis.
abstract: A group of children and adolescents with infantile nephropathic cystinosis underwent cognitive testing and were examined for cortical atrophy using magnetic resonance imaging or computed tomography. Ten of 11 patients demonstrated cortical atrophy. A consistent pattern of lower cognitive performance was found in patients with greater atrophy; however, only the relationship between atrophy and short-term memory approached statistical significance. In addition, evidence for greater impairment of visual memory than of other cognitive functions was observed. This latter observation did not appear to be related to the degree of atrophy.
authors.affiliation: Department of Neuroscience, University of California School of Medicine, La Jolla.
authors.forename: S L
authors.initials: SL
authors.lastname: Nichols
authors.forename: G A
authors.initials: GA
authors.lastname: Press
authors.forename: J A
authors.initials: JA
authors.lastname: Schneider
authors.forename: D A
authors.initials: DA
authors.lastname: Trauner
mesh_terms: D000293:Adolescent; D001284:Atrophy; D002540:Cerebral Cortex / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D003072:Cognition Disorders / Q000175:diagnosis* / Q000473:pathology; D003554:Cystinosis / Q000175:diagnosis* / Q000473:pathology; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D019965:Neurocognitive Disorders / Q000175:diagnosis* / Q000473:pathology; D009483:Neuropsychological Tests