Cargando…

A Longitudinal Follow-Up Study of Intellectual Function in Duchenne Muscular Dystrophy over Age: Is It Really Stable?

The aim of the study was to retrospectively evaluate the consistency of longitudinal findings on intellectual functioning in DMD boys and their relationship to behavioral and neuropsychiatric difficulties. The cohort included 70 patients of age 3 to 17 years with at least two assessments using the W...

Descripción completa

Detalles Bibliográficos
Autores principales: Chieffo, Daniela P. R., Moriconi, Federica, Pane, Marika, Lucibello, Simona, Ferraroli, Elisabetta, Norcia, Giulia, Ricci, Martina, Capasso, Anna, Cicala, Gianpaolo, Buchignani, Bianca, Coratti, Giorgia, Cutrona, Costanza, Pelizzari, Monia, Brogna, Claudia, Hendriksen, Jos G. M., Muntoni, Francesco, Mercuri, Eugenio
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9865074/
https://www.ncbi.nlm.nih.gov/pubmed/36675332
http://dx.doi.org/10.3390/jcm12020403
_version_ 1784875745482375168
author Chieffo, Daniela P. R.
Moriconi, Federica
Pane, Marika
Lucibello, Simona
Ferraroli, Elisabetta
Norcia, Giulia
Ricci, Martina
Capasso, Anna
Cicala, Gianpaolo
Buchignani, Bianca
Coratti, Giorgia
Cutrona, Costanza
Pelizzari, Monia
Brogna, Claudia
Hendriksen, Jos G. M.
Muntoni, Francesco
Mercuri, Eugenio
author_facet Chieffo, Daniela P. R.
Moriconi, Federica
Pane, Marika
Lucibello, Simona
Ferraroli, Elisabetta
Norcia, Giulia
Ricci, Martina
Capasso, Anna
Cicala, Gianpaolo
Buchignani, Bianca
Coratti, Giorgia
Cutrona, Costanza
Pelizzari, Monia
Brogna, Claudia
Hendriksen, Jos G. M.
Muntoni, Francesco
Mercuri, Eugenio
author_sort Chieffo, Daniela P. R.
collection PubMed
description The aim of the study was to retrospectively evaluate the consistency of longitudinal findings on intellectual functioning in DMD boys and their relationship to behavioral and neuropsychiatric difficulties. The cohort included 70 patients of age 3 to 17 years with at least two assessments using the Wechsler scales. CBCL and clinical observation of behavior were also performed. Changes in total intelligence quotient were interpreted as stable or not stable using the reliable-change method. On the first assessment 43/70 had normal quotients, 18 borderline, 5 mild, and 4 moderate intellectual disability, while 27/70 had no behavioral disorders, 17 had abnormal CBCL, and 26 patients had clear signs of attention deficits despite normal CBCL. The remaining seven were untestable. The mean total intelligence quotient change in the cohort was −2.99 points (SD: 12.29). Stable results on TIQ were found in 63% of the paired assessments. A third of the consecutive cognitive assessments showed a difference of more than 11 points with changes up to 42 points. Boys with no behavioral/attention disorder had smaller changes than those with attention (p = 0.007) and behavioral disorders (p = 0.002). Changes in IQ may occur in Duchenne and are likely to be associated with behavioral or attention deficits.
format Online
Article
Text
id pubmed-9865074
institution National Center for Biotechnology Information
language English
publishDate 2023
publisher MDPI
record_format MEDLINE/PubMed
spelling pubmed-98650742023-01-22 A Longitudinal Follow-Up Study of Intellectual Function in Duchenne Muscular Dystrophy over Age: Is It Really Stable? Chieffo, Daniela P. R. Moriconi, Federica Pane, Marika Lucibello, Simona Ferraroli, Elisabetta Norcia, Giulia Ricci, Martina Capasso, Anna Cicala, Gianpaolo Buchignani, Bianca Coratti, Giorgia Cutrona, Costanza Pelizzari, Monia Brogna, Claudia Hendriksen, Jos G. M. Muntoni, Francesco Mercuri, Eugenio J Clin Med Article The aim of the study was to retrospectively evaluate the consistency of longitudinal findings on intellectual functioning in DMD boys and their relationship to behavioral and neuropsychiatric difficulties. The cohort included 70 patients of age 3 to 17 years with at least two assessments using the Wechsler scales. CBCL and clinical observation of behavior were also performed. Changes in total intelligence quotient were interpreted as stable or not stable using the reliable-change method. On the first assessment 43/70 had normal quotients, 18 borderline, 5 mild, and 4 moderate intellectual disability, while 27/70 had no behavioral disorders, 17 had abnormal CBCL, and 26 patients had clear signs of attention deficits despite normal CBCL. The remaining seven were untestable. The mean total intelligence quotient change in the cohort was −2.99 points (SD: 12.29). Stable results on TIQ were found in 63% of the paired assessments. A third of the consecutive cognitive assessments showed a difference of more than 11 points with changes up to 42 points. Boys with no behavioral/attention disorder had smaller changes than those with attention (p = 0.007) and behavioral disorders (p = 0.002). Changes in IQ may occur in Duchenne and are likely to be associated with behavioral or attention deficits. MDPI 2023-01-04 /pmc/articles/PMC9865074/ /pubmed/36675332 http://dx.doi.org/10.3390/jcm12020403 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Chieffo, Daniela P. R.
Moriconi, Federica
Pane, Marika
Lucibello, Simona
Ferraroli, Elisabetta
Norcia, Giulia
Ricci, Martina
Capasso, Anna
Cicala, Gianpaolo
Buchignani, Bianca
Coratti, Giorgia
Cutrona, Costanza
Pelizzari, Monia
Brogna, Claudia
Hendriksen, Jos G. M.
Muntoni, Francesco
Mercuri, Eugenio
A Longitudinal Follow-Up Study of Intellectual Function in Duchenne Muscular Dystrophy over Age: Is It Really Stable?
title A Longitudinal Follow-Up Study of Intellectual Function in Duchenne Muscular Dystrophy over Age: Is It Really Stable?
title_full A Longitudinal Follow-Up Study of Intellectual Function in Duchenne Muscular Dystrophy over Age: Is It Really Stable?
title_fullStr A Longitudinal Follow-Up Study of Intellectual Function in Duchenne Muscular Dystrophy over Age: Is It Really Stable?
title_full_unstemmed A Longitudinal Follow-Up Study of Intellectual Function in Duchenne Muscular Dystrophy over Age: Is It Really Stable?
title_short A Longitudinal Follow-Up Study of Intellectual Function in Duchenne Muscular Dystrophy over Age: Is It Really Stable?
title_sort longitudinal follow-up study of intellectual function in duchenne muscular dystrophy over age: is it really stable?
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9865074/
https://www.ncbi.nlm.nih.gov/pubmed/36675332
http://dx.doi.org/10.3390/jcm12020403
work_keys_str_mv AT chieffodanielapr alongitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT moriconifederica alongitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT panemarika alongitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT lucibellosimona alongitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT ferrarolielisabetta alongitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT norciagiulia alongitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT riccimartina alongitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT capassoanna alongitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT cicalagianpaolo alongitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT buchignanibianca alongitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT corattigiorgia alongitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT cutronacostanza alongitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT pelizzarimonia alongitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT brognaclaudia alongitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT hendriksenjosgm alongitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT muntonifrancesco alongitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT mercurieugenio alongitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT chieffodanielapr longitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT moriconifederica longitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT panemarika longitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT lucibellosimona longitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT ferrarolielisabetta longitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT norciagiulia longitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT riccimartina longitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT capassoanna longitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT cicalagianpaolo longitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT buchignanibianca longitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT corattigiorgia longitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT cutronacostanza longitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT pelizzarimonia longitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT brognaclaudia longitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT hendriksenjosgm longitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT muntonifrancesco longitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable
AT mercurieugenio longitudinalfollowupstudyofintellectualfunctioninduchennemusculardystrophyoverageisitreallystable