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 Sciences

HudsonG. Taylor¹  Richard Boada²  Katherine Koenig³  InesA. Basten⁴  AlbertoC. S. Costa⁴  VeridianaL. Barrionuevo⁵  AnaC. Brandão⁵ 

[1] Center for Biobehavioral Health, Nationwide Children’s Hospital Research Institute and The Ohio State University, Columbus, OH 43205, USA;Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO 80045, USA;Department of Radiology, Cleveland Clinic Lerner College of Medicine, Cleveland, OH 44195, USA;Division of Neurology and Epilepsy, Department of Pediatrics, Case Western Reserve University, Cleveland, OH 44106, USA;Hospital Israelita Albert Einstein, São Paulo, SP, CEP 05652, Brazil;

关键词: down syndrome;    hippocampal deficits;    executive function;    episodic memory;    working memory;    neurocognitive phenotype;    memantine;    NMDA receptor;    Alzheimer’s disease;   

DOI  :  10.3390/brainsci8120205

来源: DOAJ

【 摘 要 】

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.

【 授权许可】

Unknown