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

IRCCS Santa Lucia Foundation, Via del Fosso di Fiorano, Rome, Italy.
Behavior Genetics (Impact Factor: 3.21). 05/2011; 41(3):445-56. 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.

15 Reads
  • Source
    • "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.
    Research in Developmental Disabilities 01/2015; 38C:97-107. DOI:10.1016/j.ridd.2014.11.022 · 3.40 Impact Factor
  • Source
    • "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.
    Current Alzheimer research 01/2014; 11(2). DOI:10.2174/1567205011666140130151344 · 3.89 Impact Factor
  • Source
    • "Although a high variability in cognitive and behavioral performance has been described in the WS population (Porter & Coltheart, 2005, 2006), studies on cognitive and psychopathological functioning have documented specific features in WS. Mild to severe intellectual disability co-occurs with relatively spared language, exceptional facial recognition, and severe deficits in visuo-spatial processing, exploration, executive functions and implicit learning (Atkinson et al., 2001; Bellugi, Lichtenberger, Jones, Lai, & St George, 2000; Foti et al., 2011; Mandolesi et al., 2009; Menghini, Addona, Costanzo, & Vicari, 2010; Vicari, Verucci, & Carlesimo, 2007). As for psychiatric disorders, anxiety is the most prevalent disorder in WS individuals (Dodd & Porter, 2009). "
    [Show abstract] [Hide abstract]
    ABSTRACT: In Williams syndrome (WS) cerebellar measures were only indirectly related to behavioral outcomes. T1-weighted magnetic resonance images and neuropsychological data were acquired to investigate whether cerebellar vermis differences were present in 12 WS individuals compared with 13 chronological age-matched controls and whether WS cerebellar vermis measures were related to cognitive scores. In WS participants, we observed a significant increase in the volume of the posterior superior cerebellar vermis (lobules VI-VII) and an atypical ratio between width and height of the cerebellar vermis. Furthermore, we found an inverse correlation between cerebellar posterior vermis volume and scores on implicit learning, phonological fluency and the verbal short-term memory tasks. The present study supported a role for the posterior cerebellar vermis in higher cognitive processes and indicated that the cerebellar vermis abnormalities (enlargement) in WS individuals have an effect in worsening the cognitive performance in specific domains.
    Research in developmental disabilities 04/2013; 34(7):2118-2126. DOI:10.1016/j.ridd.2013.03.026 · 4.41 Impact Factor
Show more

Similar Publications