کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
10466004 | 925747 | 2010 | 7 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Neurological soft signs feature a double dissociation within the language system in Williams syndrome
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کلمات کلیدی
WPPSIWBSELNVMIWISC-RPPVTElastin - الاستینPeabody Picture Vocabulary Test - تست واژگان تصویر PeabodyWilliams-Beuren syndrome - سندرم ویلیامز-بیورنCognition - شناختbasal ganglia - عقدههای قاعدهایFluorescent in situ hybridisation - فلورسنت در محل hybridisationFish - ماهیCerebellum - مخچهVisuospatial skills - مهارت های همه جانبهNeurological signs - نشانه های عصبیWechsler Preschool and Primary Scale of Intelligence - پیش دبستانی و مقیاس اطلاعات اولیه Wechsler
موضوعات مرتبط
علوم زیستی و بیوفناوری
علم عصب شناسی
علوم اعصاب رفتاری
پیش نمایش صفحه اول مقاله
چکیده انگلیسی
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 significant. In both instances, the effect size was greater for syntactic measures. We take these novel findings as suggestive of a double dissociation between expressive and receptive skills at sentence level within the WBS linguistic phenotype. The investigation of neuromotor soft signs and neuropsychological functions may provide a key to new non-cortico-centric genotype/phenotype relationships.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Neuropsychologia - Volume 48, Issue 11, September 2010, Pages 3298-3304
Journal: Neuropsychologia - Volume 48, Issue 11, September 2010, Pages 3298-3304
نویسندگان
Alessandro Tavano, Chiara Gagliardi, Sara Martelli, Renato Borgatti,