Cognitive Dysfunction in Down Syndrome

A special issue of Brain Sciences (ISSN 2076-3425).

Deadline for manuscript submissions: closed (15 July 2017) | Viewed by 22458

Special Issue Editor


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Guest Editor
Department Psychiatry, Case Western Reserve University, Cleveland, OH 44106, USA
Interests: down syndrome; neurophysiology; neuropsychology; preclinical research; clinical research
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

In this Special Issue of Brain Sciences, we will be discussing “Cognitive Dysfunction in Down Syndrome” from several perspectives, in an attempt to find and/or synthesize critical areas that may one day lead to the discovery of pharmacological ways of addressing some of the key components leading to developmental and intellectual disabilities associated with the trisomy of chromosome 21. We will welcome articles describing system-specific neuropsychological deficits commonly found in persons with Down syndrome; new experimental data (collected from human beings and animal models) and reviews on the neurobiology and neuropharmacology of Down syndrome; and critical reviews on the design and conduct of clinical trials in this field. We hope we can count on your valuable contribution to make this a successful and impactful Special Issue for this journal.

Prof. Dr. Alberto Costa
Guest Editor

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Keywords

  • Down syndrome
  • Neuropsychology
  • Neurobiology
  • Neuropharmacology
  • Clinical trials

Published Papers (3 papers)

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Research

201 KiB  
Article
Repetitive Behaviours and Restricted Interests in Individuals with Down Syndrome—One Way of Managing Their World?
by Sheila Glenn
Brain Sci. 2017, 7(6), 66; https://doi.org/10.3390/brainsci7060066 - 15 Jun 2017
Cited by 7 | Viewed by 4707
Abstract
This paper argues that the repetitive behaviour and restrictive interests (RBRI) displayed by individuals with Down syndrome have mostly positive functions. However, as research has developed from interests in Obsessional Compulsive Disorder or Autistic Spectrum Disorder, unfortunately a view has arisen that RBRI [...] Read more.
This paper argues that the repetitive behaviour and restrictive interests (RBRI) displayed by individuals with Down syndrome have mostly positive functions. However, as research has developed from interests in Obsessional Compulsive Disorder or Autistic Spectrum Disorder, unfortunately a view has arisen that RBRI in individuals with Down syndrome are also likely to be pathological. This is particularly the case in adults. The paper reviews: (a) measures employed and the perspectives that have been used; (b) the development in typically developing individuals, those with Down syndrome, and those with other conditions associated with intellectual disability; (c) positive and possible negative effects of RBRI; and (d) the need for more research. The conclusion is that, for their level of development, RBRI are helpful for most individuals with Down syndrome. Full article
(This article belongs to the Special Issue Cognitive Dysfunction in Down Syndrome)
236 KiB  
Article
Acute Regression in Young People with Down Syndrome
by Clotilde Mircher, Cécile Cieuta-Walti, Isabelle Marey, Anne-Sophie Rebillat, Laura Cretu, Eliane Milenko, Martine Conte, Franck Sturtz, Marie-Odile Rethore and Aimé Ravel
Brain Sci. 2017, 7(6), 57; https://doi.org/10.3390/brainsci7060057 - 27 May 2017
Cited by 48 | Viewed by 10620
Abstract
Abstract: Adolescents and young adults with Down syndrome (DS) can present a rapid regression with loss of independence and daily skills. Causes of regression are unknown and treatment is most of the time symptomatic. We did a retrospective cohort study of regression [...] Read more.
Abstract: Adolescents and young adults with Down syndrome (DS) can present a rapid regression with loss of independence and daily skills. Causes of regression are unknown and treatment is most of the time symptomatic. We did a retrospective cohort study of regression cases: patients were born between 1959 and 2000, and were followed from 1984 to now. We found 30 DS patients aged 11 to 30 years old with history of regression. Regression occurred regardless of the cognitive level (severe, moderate, or mild intellectual disability (ID)). Patients presented psychiatric symptoms (catatonia, depression, delusions, stereotypies, etc.), partial or total loss of independence in activities of daily living (dressing, toilet, meals, and continence), language impairment (silence, whispered voice, etc.), and loss of academic skills. All patients experienced severe emotional stress prior to regression, which may be considered the trigger. Partial or total recovery was observed for about 50% of them. In our cohort, girls were more frequently affected than boys (64%). Neurobiological hypotheses are discussed as well as preventative and therapeutic approaches. Full article
(This article belongs to the Special Issue Cognitive Dysfunction in Down Syndrome)
2142 KiB  
Article
Emotion Recognition in Adolescents with Down Syndrome: A Nonverbal Approach
by Régis Pochon, Claire Touchet and Laure Ibernon
Brain Sci. 2017, 7(6), 55; https://doi.org/10.3390/brainsci7060055 - 23 May 2017
Cited by 20 | Viewed by 6679
Abstract
Several studies have reported that persons with Down syndrome (DS) have difficulties recognizing emotions; however, there is insufficient research to prove that a deficit of emotional knowledge exists in DS. The aim of this study was to evaluate the recognition of emotional facial [...] Read more.
Several studies have reported that persons with Down syndrome (DS) have difficulties recognizing emotions; however, there is insufficient research to prove that a deficit of emotional knowledge exists in DS. The aim of this study was to evaluate the recognition of emotional facial expressions without making use of emotional vocabulary, given the language problems known to be associated with this syndrome. The ability to recognize six emotions was assessed in 24 adolescents with DS. Their performance was compared to that of 24 typically developing children with the same nonverbal-developmental age, as assessed by Raven’s Progressive Matrices. Analysis of the results revealed no global difference; only marginal differences in the recognition of different emotions appeared. Study of the developmental trajectories revealed a developmental difference: the nonverbal reasoning level assessed by Raven’s matrices did not predict success on the experimental tasks in the DS group, contrary to the typically developing group. These results do not corroborate the hypothesis that there is an emotional knowledge deficit in DS and emphasize the importance of using dynamic, strictly nonverbal tasks in populations with language disorders. Full article
(This article belongs to the Special Issue Cognitive Dysfunction in Down Syndrome)
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