Neurological soft signs feature a double dissociation within the language system in Williams syndrome

Neuropsychologia
Alessandro TavanoRenato Borgatti

Abstract

The neurocognitive profile of Williams-Beuren syndrome (WBS) is characterized by visuospatial deficits, apparently fluent language, motor soft signs, and hypersociability. We investigated the association between neuromotor soft signs and visuospatial, executive-attentive, mnestic and linguistic functions in a group of 26 children and young adults with WBS. We hypothesized that neurological soft signs could be an index of subtle neurofunctional deficits and thus provide a behavioural window into the processes underlying neurocognition in Williams-Beuren syndrome. Dysmetria and dystonic movements were selected as grouping neurological variables, indexing cerebellar and basal ganglia dysfunction, respectively. No detrimental effects on visuospatial/visuoconstructive skills were evident following the presence of either neurological variable. As for language skills, participants with dysmetria showed markedly reduced expressive syntactic and lexico-semantic skills as compared to non-affected individuals, while no difference in chronological age was evident. Participants with dystonic movements showed reduced receptive syntax and increased lexical comprehension skills as compared to non-affected individuals, the age factor being sign...Continue Reading

References

Aug 1, 1988·The Journal of Pediatrics·C A MorrisB L Blackburn
Apr 1, 1994·Journal of Clinical and Experimental Neuropsychology·P P Wang, U Bellugi
Jan 1, 1996·Journal of Child Neurology·C A ChapmanB R Pober
Jun 23, 2000·Journal of Neurology, Neurosurgery, and Psychiatry·M G LeggioM Molinari
Aug 23, 2000·Journal of Cognitive Neuroscience·U BellugiM St George
Aug 23, 2000·Journal of Cognitive Neuroscience·A L ReissU Bellugi
Aug 23, 2000·Journal of Cognitive Neuroscience·J R KorenbergR Matsuoka
Mar 27, 2001·Developmental Medicine and Child Neurology·J E SchmittA L Reiss
Jun 29, 2002·Journal of Child Neurology·Petter StrømmeKjersti Ramstad
Jul 12, 2002·Journal of Child Language·Julia GrantAnnette Karmiloff-Smith
Nov 7, 2002·Developmental Medicine and Child Neurology·Wendy JonesUrsula Bellugi
Feb 5, 2003·Journal of Medical Genetics·A Karmiloff-SmithM Tassabehji
Feb 20, 2003·Neuropsychologia·Chiara GagliardiRenato Borgatti
Jan 7, 2004·American Journal of Medical Genetics. Part a·Teresa F DoyleJohn Graham
Jan 24, 2004·Journal of the International Neuropsychological Society : JINS·Sonja A KotzAngela D Friederici
Apr 9, 2004·Cortex; a Journal Devoted to the Study of the Nervous System and Behavior·Caroline E PhillipsAnnette Karmiloff-Smith
Sep 2, 2004·Neuron·Andreas Meyer-LindenbergKaren Faith Berman
Sep 21, 2004·The Journal of Neuropsychiatry and Clinical Neurosciences·Jeremy D Schmahmann
Nov 10, 2004·American Journal of Medical Genetics. Part a·Elizabeth M CherniskeBarbara R Pober
Jan 8, 2005·Journal of the International Neuropsychological Society : JINS·Stefano VicariVirginia Volterra
Feb 25, 2005·Neuroreport·Marco TettamantiDaniela Perani
Apr 22, 2005·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Paul M ThompsonAllan L Reiss
Jun 14, 2005·The Journal of Clinical Investigation·Andreas Meyer-LindenbergKaren Faith Berman
Jul 12, 2005·Neuropsychologia·Elisa FrigerioRenato Borgatti
Jul 12, 2005·Nature Neuroscience·Andreas Meyer-LindenbergKaren Faith Berman
Aug 27, 2005·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·J Shane KippenhanKaren Faith Berman
Oct 26, 2005·Brain and Language·Timothy JustusRichard B Ivry
Mar 1, 1978·Cortex; a Journal Devoted to the Study of the Nervous System and Behavior·E De Renzi, P Faglioni
Jun 9, 2006·Nature Reviews. Neuroscience·Andreas Meyer-LindenbergKaren Faith Berman
Jul 1, 2006·Cellular and Molecular Life Sciences : CMLS·M A EckertA L Reiss
Dec 22, 2006·Developmental Science·Annette Karmiloff-Smith
Feb 28, 2007·Mental Retardation and Developmental Disabilities Research Reviews·Carolyn B Mervis, Angela M Becerra
May 19, 2007·Pediatric Neurology·Chiara GagliardiRenato Borgatti
Sep 11, 2007·Proceedings of the National Academy of Sciences of the United States of America·Stefano MarencoKaren Faith Berman
Sep 18, 2007·Brain : a Journal of Neurology·Alessandro TavanoRenato Borgatti
Oct 30, 2007·Proceedings of the National Academy of Sciences of the United States of America·Luigi CattaneoGiacomo Rizzolatti
Nov 3, 2007·The American Journal of Psychiatry·B J CaseyGary Glover
Nov 10, 2007·Journal of Intellectual Disability Research : JIDR·S VicariG A Carlesimo
Nov 17, 2007·Neuroscience and Biobehavioral Reviews·Darren R HockingNicole J Rinehart

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Citations

Mar 19, 2013·Research in Developmental Disabilities·Floriana CostanzoStefano Vicari
Mar 14, 2012·American Journal of Medical Genetics. Part a·Stefania BarozziDaniele Brambilla
Dec 7, 2013·Asian Journal of Psychiatry·Khaled SaadReda Badry
Jun 9, 2021·Applied Neuropsychology. Child·Deisiane Oliveira SoutoVitor Geraldi Haase
Jun 4, 2021·Current Opinion in Genetics & Development·Michael Mortillo, Jennifer G Mulle

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