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Neuropsychological Phenotype in Wolf-Hirschhorn Syndrome
ISSN: 2292 - 2598
Publisher: author
Neuropsychological Phenotype in Wolf-Hirschhorn Syndrome
Indexed in
Medical Sciences
ARTICLE-FACTOR
1.3
Article Basics Score: 2
Article Transparency Score: 3
Article Operation Score: 2
Article Articles Score: 2
Article Accessibility Score: 2
SUBMIT PAPER ASK QUESTION
International Category Code (ICC):
ICC-1702
Publisher: Lifescience Global Inc.
International Journal Address (IAA):
IAA.ZONE/2292110702598
eISSN
:
2292 - 2598
VALID
ISSN Validator
Abstract
The Wolf-Hirschhorn syndrome (WHS) is a rare genetic disorder that causes a range of intellectual disability from mild to severe. In this study, we used standard tools to psychometrically characterize the specific neuropsychological phenotype of WHS. We studied 57 individuals with WHS, ranging in age from 2.6 to 28.6 years representing 70% of the certified Italian WHS population. Results obtained by administering Griffiths' Mental Developmental Scales and the Vineland Adaptive Behavior Scale revealed a typical WHS neuropsychological phenotype characterized by specific strengths and weaknesses. Despite their severe cognitive impairment, in both scales, patients showed better communication and social interaction skills compared to visuo-motor abilities. Results of our study could bring to the development of new and more effective treatments for individuals affected by WHS: based on neuropsychological phenotype description, it should be ...
