Spatial Competences in Prader–Willi Syndrome: A Radial Arm Maze Study

ArticleinBehavior Genetics 41(3):445-56 · May 2011with23 Reads
DOI: 10.1007/s10519-011-9471-4 · Source: PubMed
The present study was aimed at investigating the spatial abilities in Prader-Willi syndrome (PWS) by using the Radial Arm Maze (RAM) task. We trained PWS individuals with the deletion subtype in two different RAM paradigms that tapped different aspects of spatial memory. To evaluate the extent of spatial deficit in PWS individuals, it seemed interesting to compare their performances with those of individuals with Williams syndrome (WS) in which deficits in spatial abilities have been well described. The two syndromic groups were compared to typically developing (TD) individuals mental-age and gender matched. The findings evidenced the impairment of PWS individuals in solving the RAM task with variable severity depending on the paradigm requests. Since the RAM is a task that allows the acquisition of spatial competences through the movement, we advance that the spatial deficits observed in PWS individuals may be related to the malfunctioning of spatial and motor integrative processing.
    • "Indeed, the visuo-spatial domain is a strength point in PWS and conversely a strong weakness in WS. The WS deficits in spatial working and long-term memory [10,5152535455 heavily impaired performances in all EPs. Finally, the PWS performances harmonize with the good capacity of spatial learning and localizatory memory shown by an animal model deficient of Necdin, a candidate gene in PWS etiology [56].Figure 4 Incorrect items touched on the screen by PWS, WS, and TD participants in performing the tasks. "
    [Show abstract] [Hide abstract] ABSTRACT: New competencies may be learned through active experience (learning by doing) or observation of others’ experience (learning by observation). Observing another person performing a complex action accelerates the observer’s acquisition of the same action, limiting the time-consuming process of learning by doing. Here, we compared learning by observation and learning by doing in individuals with Prader-Willi syndrome (PWS). It is hypothesized that PWS individuals could show more difficulties with learning by observation than learning by doing because of their specific difficulty in interpreting and using social information. The performance of 24 PWS individuals was compared with that of 28 mental age (MA)- and gender-matched typically developing (TD) children in tasks of learning a visuo-motor sequence by observation or by doing. To determine whether the performance pattern exhibited by PWS participants was specific to this population or whether it was a nonspecific intellectual disability effect, we compared the PWS performances with those of a third MA- and gender-matched group of individuals with Williams syndrome (WS). PWS individuals were severely impaired in detecting a sequence by observation, were able to detect a sequence by doing, and became as efficient as TD children in reproducing an observed sequence after a task of learning by doing. The learning pattern of PWS children was reversed compared with that of WS individuals. The observational learning deficit in PWS individuals may be rooted, at least partially, in their incapacity to understand and/or use social information.
    Full-text · Article · Feb 2015
    • "To determine whether the performance exhibited by PWS participants was specific to this population or whether it was a non-specific effect of the Intellectual Disability (ID), we compared PWS performances with those of a mental age-and gender-matched group of individuals with Williams syndrome (WS) exhibiting a specific impairment of the visuo-spatial abilities (Foti, Petrosini, et al., 2011; Mandolesi, Addona, et al., 2009; Vicari, Bellucci, & Carlesimo, 2006). The performances of PWS and WS individuals were compared with those of a third mental age-and gender-matched group of typically developing (TD) children used as a nonsyndromic control. "
    [Show abstract] [Hide abstract] ABSTRACT: This study was aimed at evaluating the spatial abilities in individuals with Prader-Willi syndrome (PWS) by using an ecological large-scale task with multiple rewards. To evaluate the extent of spatial deficit in PWS individuals, we compare their performances with those of individuals with Williams Syndrome (WS) in which the spatial deficits have been widely described. Participants had to explore an open space to search nine rewards placed in buckets arranged according to three spatial configurations: a Cross, a 3×3 Matrix and a Cluster composed by three groups of three buckets each. PWS individuals exhibited an explorative deficit in Cluster and Cross configurations, while WS participants in Matrix and Cross configurations. The findings indicate that the structural affordances of the environment influence the explorative strategies and can be related to how spatial information is processed. Copyright © 2014 Elsevier Ltd. All rights reserved.
    Full-text · Article · Jan 2015
    F FotiF FotiD MenghiniD MenghiniL PetrosiniL Petrosini+1more author...[...]
    • "The Radial Arm Maze (RAM) is a practicable memory test to determine the effect of drugs. Foti et al. [16] found that most aspects of spatial function can be analysed by movement in the RAM, in addition to analytical memory and appropriate working memory. "
    [Show abstract] [Hide abstract] ABSTRACT: Aquilaria subintegra, locally known as "Gaharu", belongs to the Thymelaeceae family. This plant's leaves have been claimed to be effective for the treatment of Alzheimer's disease (AD) by Malay traditional practitioner in Malaysia. In this research, the chloroform extracts of the leaves and stem of A. subintegra were tested for acetylcholinesterase (AChE) inhibitory activity. The Thin Layer Chromatography (TLC) results indicated the presence of phenols, flavonoids, terpenoids, and alkaloids compounds in the extracts. Analysis of the stem chloroform extracts with LCMS/MS displayed that it contains kaempferol 3,4,7-trimethyl ether. The AChE inhibitory activity of leaves and stem chloroform extracts and kaempferol were 80%, 93% and 85.8%, respectively. The Brine Shrimp Lethality Assay (BSLA) exhibited low to moderate toxicity of the chloroform extract from leaves (LC50=531.18 ± 49.53 µg/ml), the stem chloroform extract (LC50=407.34 ± 68.05 µg/ml) and kaempferol (LC50=762.41 ± 45.09 µg/ml). The extracts and kaempferol were not cytotoxic to human umbilical vein endothelial cells (HUVEC), human normal gastric epithelial cell line (GES-1) and human normal hepatic cell line (WRL-68). The effect of leaf and stem chloroform extracts and kaempferol were determined in the Radial Arm Maze (RAM) after administration by oral gavage to ICR male and female mice with valium-impaired memory. Administration of kaempferol to the mice significantly reduced the number of repeated entries into the arms of maze in males and females. In conclusion, the inhibition of AChE by leaf and stem chloroform extracts of A. subintegra could be due to the presence of kaempferol. This extract is safe for use as a natural AChE inhibitor as an alternative to berberine for the treatment of AD.
    Full-text · Article · Jan 2014
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