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Brain Sci. 2018, 8(12), 205; https://doi.org/10.3390/brainsci8120205

On the Design of Broad-Based Neuropsychological Test Batteries to Assess the Cognitive Abilities of Individuals with Down Syndrome in the Context of Clinical Trials

1
Division of Neurology and Epilepsy, Department of Pediatrics, Case Western Reserve University, Cleveland, OH 44106, USA
2
Department of Child and Adolescent Psychiatry, Universitair Ziekenhuis Brussel (UZ Brussel), B-1090 Brussels, Belgium
3
Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO 80045, USA
4
Children’s Hospital Colorado, Aurora, CO 80045, USA
5
Center for Biobehavioral Health, Nationwide Children’s Hospital Research Institute and The Ohio State University, Columbus, OH 43205, USA
6
Division of Developmental/Behavioral Pediatrics and Psychology, Department of Pediatrics, Case Western Reserve University, Cleveland, OH 44106, USA
7
Department of Radiology, Cleveland Clinic Lerner College of Medicine, Cleveland, OH 44195, USA
8
Mellen Center, The Cleveland Clinic, Cleveland, OH 44195, USA
9
Hospital Israelita Albert Einstein, São Paulo, SP, CEP 05652, Brazil
10
Department of Psychiatry, Case Western Reserve University, Cleveland, OH 44106, USA
*
Author to whom correspondence should be addressed.
Received: 16 October 2018 / Revised: 14 November 2018 / Accepted: 21 November 2018 / Published: 26 November 2018
(This article belongs to the Special Issue Research on Down Syndrome)
Full-Text   |   PDF [301 KB, uploaded 26 November 2018]

Abstract

Down syndrome (DS) is the most common genetically-defined cause of intellectual disability. Neurodevelopmental deficits displayed by individuals with DS are generally global, however, disproportionate deficits in cognitive processes that depend heavily on the hippocampus and prefrontal cortex are also well documented. Additionally, DS is associated with relative strengths in visual processing and visuospatial short-term memory, and weaknesses in the verbal domain. Although reports of pharmacological rescuing of learning and memory deficits in mouse models of DS abound in the literature, proving the principle that cognitive ability of persons with DS can be boosted through pharmacological means is still an elusive goal. The design of customized batteries of neuropsychological efficacy outcome measures is essential for the successful implementation of clinical trials of potential cognitive enhancing strategies. Here, we review the neurocognitive phenotype of individuals with DS and major broad-based test batteries designed to quantify specific cognitive domains in these individuals, including the one used in a pilot trial of the drug memantine. The main goal is to illustrate the essential considerations in planning trials to enhance cognitive functions in individuals with DS, which should also have implications for the design of similar studies in individuals with other forms of intellectual disability. View Full-Text
Keywords: down syndrome; hippocampal deficits; executive function; episodic memory; working memory; neurocognitive phenotype; memantine; NMDA receptor; Alzheimer’s disease down syndrome; hippocampal deficits; executive function; episodic memory; working memory; neurocognitive phenotype; memantine; NMDA receptor; Alzheimer’s disease
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited (CC BY 4.0).
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Basten, I.A.; Boada, R.; Taylor, H.G.; Koenig, K.; Barrionuevo, V.L.; Brandão, A.C.; Costa, A.C.S. On the Design of Broad-Based Neuropsychological Test Batteries to Assess the Cognitive Abilities of Individuals with Down Syndrome in the Context of Clinical Trials. Brain Sci. 2018, 8, 205.

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