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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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