Please use this identifier to cite or link to this item: https://hdl.handle.net/11499/6654
Title: Cognitive functions in neurofibromatosis type 1 patients and unaffected siblings
Authors: Erdogan-Bakar, E.
Cinbiş, Mine
Özyürek, H.
Kiriş, N.
Altunbaşak, Ş.
Anlar, B.
Keywords: Cognitive
Learning
Mental
Neurofibromatosis type 1
NF1
adolescent
adult
arithmetic
article
attention deficit disorder
child
cognition
controlled study
environmental factor
female
heredity
human
intelligence
major clinical study
male
mental deficiency
neurofibromatosis
prevalence
school child
sibling
vision
visuomotor coordination
Wechsler Intelligence Scale
Adolescent
Child
Cognition
Female
Follow-Up Studies
Humans
Male
Neurofibromatosis 1
Orientation
Severity of Illness Index
Siblings
Visual Perception
Young Adult
Abstract: Attention, learning, and perceptual problems have been reported at various degrees and rates in neurofibromatosis type 1 (NF1). We aimed to define the cognitive profiles frequently associated with NF1. Children and adolescents with NF1 (n=58) were tested using Wechsler Intelligence Scales for Children-Revised (WISC-R), Judgment of Line Orientation, and Bender Visual-Motor Gestalt tests. Comparison groups were unaffected siblings of NF1 patients (n=20), children with attention deficit and hyperactivity disorder (ADHD, n=40), and normal children (n=40). No difference was found between familial or sporadic NF1 cases. Seventeen/58 (29%) of NF1 cases had a full scale IQ <70. The subgroup of NF1 patients with full scale IQ>80 (n=27) scored lower in WISC-R subtests measuring visual perception when compared to a healthy control group of similar intelligence, and lower in arithmetic but better in Bender-Gestalt and Judgment of Line Orientation tests when compared to an ADHD group of similar intelligence. These results indicate a high prevalence of mental retardation in a clinical NF1 series. NF1 patients who have normal intelligence may have impaired visual perception, but their visual perceptual problems are less than in ADHD. The tendency of unaffected siblings of NF1 patients to have mildly but consistently low test scores compared to healthy controls needs to be studied further for underlying genetic or environmental factors.
URI: https://hdl.handle.net/11499/6654
ISSN: 0041-4301
Appears in Collections:PubMed İndeksli Yayınlar Koleksiyonu / PubMed Indexed Publications Collection
Scopus İndeksli Yayınlar Koleksiyonu / Scopus Indexed Publications Collection
Tıp Fakültesi Koleksiyonu
WoS İndeksli Yayınlar Koleksiyonu / WoS Indexed Publications Collection

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