
Fulltext:
174236.pdf
Embargo:
until further notice
Size:
62.85Kb
Format:
PDF
Description:
Publisher’s version
Publication year
2017Number of pages
2 p.
Source
European Psychiatry, 41, , (2017), pp. S157-S158ISSN
Publication type
Article / Letter to editor

Display more detailsDisplay less details
Organization
SW OZ DCC NRP
Journal title
European Psychiatry
Volume
vol. 41
Issue
iss.
Languages used
English (eng)
Page start
p. S157
Page end
p. S158
Subject
DI-BCB_DCC_Theme 3: Plasticity and Memory; Neuropsychology and rehabilitation psychology; Neuro- en revalidatiepsychologieAbstract
Objective: KBG syndrome is caused by a mutation in the ANKRD11 gene, characterized by short stature and specific dental, craniofacial and skeletal anomalies. Scarce literature on the phenotypical presentation mention delayed speech and motor development as well as mild to moderate intellectual disabilities. As to psychopathology, often, autism and ADHD are mentioned but not yet substantiated in terms of neurocognitive variables. Aim: Aim of the current study was to investigate neurocognitive aspects of KBG syndrome. Participants and Methods: Seventeen patients (aged 6–66 years; ten females) with a proven ANKRD11 mutation were compared with two different groups of patients with a genetic disorder and similar developmental ages (n = 14 and n = 10). Neuropsychological assessment was performed focusing on the level of intellectual functioning and on attention, memory, executive functioning, and social cognition. Results: In KBG patients, mild to moderate intellectual disabilities (WAIS IV Total IQ = 63.5 ± 10.7, range: 45-84) were established with a mental age that was lower than mean chronological age (6.4 ± 2.6 years versus 11 ± 5.7 years, respectively). When compared to both control groups, results indicated a relatively strong processing speed and social cognitive functioning of patients with KBG while direct recall of auditory memory was relatively poor most probably due to attentional dysfunction. Conclusions: The cognitive profile of this group of 17 patients with KBG is characterized by mild intellectual disability and diminished sustained attention in verbal tasks. Implications for diagnostic procedures and clinical management of the syndrome are discussed, also with regard to the question how this relates to classificatory diagnosis of ADHD.
This item appears in the following Collection(s)
- Academic publications [202802]
- Electronic publications [100870]
- Faculty of Social Sciences [27107]
Upload full text
Use your RU credentials (u/z-number and password) to log in with SURFconext to upload a file for processing by the repository team.