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Longitudinal data of neuropsychological profile in a cohort of Duchenne muscular dystrophy boys without cognitive impairment.
- Source :
-
Neuromuscular disorders : NMD [Neuromuscul Disord] 2021 Apr; Vol. 31 (4), pp. 319-327. Date of Electronic Publication: 2021 Feb 04. - Publication Year :
- 2021
-
Abstract
- The aim of the study was to re-assess neuropsychological profile in a group of boys with Duchenne muscular dystrophy without intellectual disability and neuropsychiatric disorder three years apart from a previous evaluation, to establish possible changes over time. We were also interested in defining more in detail correlation between genotype and neuropsychological phenotype. Thirty-three of the previous 40 subjects (mean age at follow up: 10 years and 7 months) agreed to participate in the follow up study and to perform the new assessment. The results confirm a typical neuropsychological profile, with difficulty in the manipulation of stored information, poor abstract reasoning and planning capacity and impulsiveness, supporting the involvement of a cerebellar striatal cortical network for these children. The more detailed description of subgroups of subjects, according to the real expression of Dp140, let to reveal possible genotype-neuropsychological phenotype correlations, and a more general neuropsychological impairment emerged in boys without Dp140 expression.<br /> (Copyright © 2021 Elsevier B.V. All rights reserved.)
Details
- Language :
- English
- ISSN :
- 1873-2364
- Volume :
- 31
- Issue :
- 4
- Database :
- MEDLINE
- Journal :
- Neuromuscular disorders : NMD
- Publication Type :
- Academic Journal
- Accession number :
- 33658162
- Full Text :
- https://doi.org/10.1016/j.nmd.2021.01.011