Early Language Impairment as an Integral Part of the Cognitive Phenotype in Huntington's Disease
ABSTRACT Objective Huntington's disease (HD) speech/language disorders have typically been attributed to motor and executive impairment due to striatal dysfunction. In‐depth study of linguistic skills and the role of extrastriatal structures in HD is scarce. This study aimed to explore the prof...
Saved in:
| Main Authors: | , , , , , , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2025-06-01
|
| Series: | Annals of Clinical and Translational Neurology |
| Subjects: | |
| Online Access: | https://doi.org/10.1002/acn3.70022 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1849334064777003008 |
|---|---|
| author | Arnau Puig‐Davi Carla Franch‐Marti Iñigo Ruiz‐Barrio Frederic Sampedro Jesus Perez‐Perez Jordi A. Matias‐Guiu Fernando Cuetos Gonzalo Olmedo‐Saura Laura Perez‐Carasol Andrea Horta‐Barba Ignacio Aracil‐Bolaños Javier Pagonabarraga Jaime Kulisevsky Saul Martinez‐Horta |
| author_facet | Arnau Puig‐Davi Carla Franch‐Marti Iñigo Ruiz‐Barrio Frederic Sampedro Jesus Perez‐Perez Jordi A. Matias‐Guiu Fernando Cuetos Gonzalo Olmedo‐Saura Laura Perez‐Carasol Andrea Horta‐Barba Ignacio Aracil‐Bolaños Javier Pagonabarraga Jaime Kulisevsky Saul Martinez‐Horta |
| author_sort | Arnau Puig‐Davi |
| collection | DOAJ |
| description | ABSTRACT Objective Huntington's disease (HD) speech/language disorders have typically been attributed to motor and executive impairment due to striatal dysfunction. In‐depth study of linguistic skills and the role of extrastriatal structures in HD is scarce. This study aimed to explore the profile of language compromise in HD and identify the structural neuroimaging correlates. Methods Language and structural correlates were assessed using the Mini Linguistic State Examination (MLSE) in 81 participants (20 HD‐ISS 0‐1, 40 HD‐ISS 2‐3 and 21 controls). Clinical and global cognition measures were also obtained. Imaging data included computed gray matter volume (GMV) and cortical thickness (CTh) values extracted from a general linear model with the MLSE. Correlation analyses were performed with the language components of the MLSE. Multivariate regression analyses were used to explore the predictive ability of the language components on GMV and CTh loss. Results HD individuals showed impaired MLSE performance (84.5 ± 12.8), particularly in syntax, motor speech, and to a lesser extent, semantics and phonology. Significant associations were found between linguistic performance and the structural integrity of nodes within the temporo‐parietal, fronto‐parietal, and fronto‐striatal lexical‐semantic and syntactic networks. Correlation analyses linked motor speech and syntax with predominantly left fronto‐striatal GMV and CTh clusters, while semantics had a bilateral fronto‐parietal topography. Multivariate regression analyses showed language domains as independent contributing factors of GMV and CTh loss in classical language‐related regions. Interpretation Language impairment is an integral part of the HD cognitive phenotype, with severity associated with structural disintegration in extensive cortico‐subcortical territories involved in language production and processing. |
| format | Article |
| id | doaj-art-0bc537a5b55d4272bcc8c4d945d7ac0f |
| institution | Kabale University |
| issn | 2328-9503 |
| language | English |
| publishDate | 2025-06-01 |
| publisher | Wiley |
| record_format | Article |
| series | Annals of Clinical and Translational Neurology |
| spelling | doaj-art-0bc537a5b55d4272bcc8c4d945d7ac0f2025-08-20T03:45:40ZengWileyAnnals of Clinical and Translational Neurology2328-95032025-06-011261201121310.1002/acn3.70022Early Language Impairment as an Integral Part of the Cognitive Phenotype in Huntington's DiseaseArnau Puig‐Davi0Carla Franch‐Marti1Iñigo Ruiz‐Barrio2Frederic Sampedro3Jesus Perez‐Perez4Jordi A. Matias‐Guiu5Fernando Cuetos6Gonzalo Olmedo‐Saura7Laura Perez‐Carasol8Andrea Horta‐Barba9Ignacio Aracil‐Bolaños10Javier Pagonabarraga11Jaime Kulisevsky12Saul Martinez‐Horta13Institute of Neuroscience Universitat Autònoma de Barcelona (UAB) Bellaterra SpainMovement Disorders Unit, Neurology Department Hospital de la Santa Creu i Sant Pau Barcelona SpainMovement Disorders Unit, Neurology Department Hospital de la Santa Creu i Sant Pau Barcelona SpainMovement Disorders Unit, Neurology Department Hospital de la Santa Creu i Sant Pau Barcelona SpainInstitute of Neuroscience Universitat Autònoma de Barcelona (UAB) Bellaterra SpainDepartment of Neurology Hospital Clinico San Carlos, San Carlos Institute for Health Research (IdiSSC). Universidad Complutense Madrid SpainFaculty of Psychology University of Oviedo Oviedo SpainMovement Disorders Unit, Neurology Department Hospital de la Santa Creu i Sant Pau Barcelona SpainMovement Disorders Unit, Neurology Department Hospital de la Santa Creu i Sant Pau Barcelona SpainMovement Disorders Unit, Neurology Department Hospital de la Santa Creu i Sant Pau Barcelona SpainMovement Disorders Unit, Neurology Department Hospital de la Santa Creu i Sant Pau Barcelona SpainMovement Disorders Unit, Neurology Department Hospital de la Santa Creu i Sant Pau Barcelona SpainInstitute of Neuroscience Universitat Autònoma de Barcelona (UAB) Bellaterra SpainInstitute of Neuroscience Universitat Autònoma de Barcelona (UAB) Bellaterra SpainABSTRACT Objective Huntington's disease (HD) speech/language disorders have typically been attributed to motor and executive impairment due to striatal dysfunction. In‐depth study of linguistic skills and the role of extrastriatal structures in HD is scarce. This study aimed to explore the profile of language compromise in HD and identify the structural neuroimaging correlates. Methods Language and structural correlates were assessed using the Mini Linguistic State Examination (MLSE) in 81 participants (20 HD‐ISS 0‐1, 40 HD‐ISS 2‐3 and 21 controls). Clinical and global cognition measures were also obtained. Imaging data included computed gray matter volume (GMV) and cortical thickness (CTh) values extracted from a general linear model with the MLSE. Correlation analyses were performed with the language components of the MLSE. Multivariate regression analyses were used to explore the predictive ability of the language components on GMV and CTh loss. Results HD individuals showed impaired MLSE performance (84.5 ± 12.8), particularly in syntax, motor speech, and to a lesser extent, semantics and phonology. Significant associations were found between linguistic performance and the structural integrity of nodes within the temporo‐parietal, fronto‐parietal, and fronto‐striatal lexical‐semantic and syntactic networks. Correlation analyses linked motor speech and syntax with predominantly left fronto‐striatal GMV and CTh clusters, while semantics had a bilateral fronto‐parietal topography. Multivariate regression analyses showed language domains as independent contributing factors of GMV and CTh loss in classical language‐related regions. Interpretation Language impairment is an integral part of the HD cognitive phenotype, with severity associated with structural disintegration in extensive cortico‐subcortical territories involved in language production and processing.https://doi.org/10.1002/acn3.70022brain structurecognitive phenotypeHuntington's diseaselanguageneuroimaging |
| spellingShingle | Arnau Puig‐Davi Carla Franch‐Marti Iñigo Ruiz‐Barrio Frederic Sampedro Jesus Perez‐Perez Jordi A. Matias‐Guiu Fernando Cuetos Gonzalo Olmedo‐Saura Laura Perez‐Carasol Andrea Horta‐Barba Ignacio Aracil‐Bolaños Javier Pagonabarraga Jaime Kulisevsky Saul Martinez‐Horta Early Language Impairment as an Integral Part of the Cognitive Phenotype in Huntington's Disease Annals of Clinical and Translational Neurology brain structure cognitive phenotype Huntington's disease language neuroimaging |
| title | Early Language Impairment as an Integral Part of the Cognitive Phenotype in Huntington's Disease |
| title_full | Early Language Impairment as an Integral Part of the Cognitive Phenotype in Huntington's Disease |
| title_fullStr | Early Language Impairment as an Integral Part of the Cognitive Phenotype in Huntington's Disease |
| title_full_unstemmed | Early Language Impairment as an Integral Part of the Cognitive Phenotype in Huntington's Disease |
| title_short | Early Language Impairment as an Integral Part of the Cognitive Phenotype in Huntington's Disease |
| title_sort | early language impairment as an integral part of the cognitive phenotype in huntington s disease |
| topic | brain structure cognitive phenotype Huntington's disease language neuroimaging |
| url | https://doi.org/10.1002/acn3.70022 |
| work_keys_str_mv | AT arnaupuigdavi earlylanguageimpairmentasanintegralpartofthecognitivephenotypeinhuntingtonsdisease AT carlafranchmarti earlylanguageimpairmentasanintegralpartofthecognitivephenotypeinhuntingtonsdisease AT inigoruizbarrio earlylanguageimpairmentasanintegralpartofthecognitivephenotypeinhuntingtonsdisease AT fredericsampedro earlylanguageimpairmentasanintegralpartofthecognitivephenotypeinhuntingtonsdisease AT jesusperezperez earlylanguageimpairmentasanintegralpartofthecognitivephenotypeinhuntingtonsdisease AT jordiamatiasguiu earlylanguageimpairmentasanintegralpartofthecognitivephenotypeinhuntingtonsdisease AT fernandocuetos earlylanguageimpairmentasanintegralpartofthecognitivephenotypeinhuntingtonsdisease AT gonzaloolmedosaura earlylanguageimpairmentasanintegralpartofthecognitivephenotypeinhuntingtonsdisease AT lauraperezcarasol earlylanguageimpairmentasanintegralpartofthecognitivephenotypeinhuntingtonsdisease AT andreahortabarba earlylanguageimpairmentasanintegralpartofthecognitivephenotypeinhuntingtonsdisease AT ignacioaracilbolanos earlylanguageimpairmentasanintegralpartofthecognitivephenotypeinhuntingtonsdisease AT javierpagonabarraga earlylanguageimpairmentasanintegralpartofthecognitivephenotypeinhuntingtonsdisease AT jaimekulisevsky earlylanguageimpairmentasanintegralpartofthecognitivephenotypeinhuntingtonsdisease AT saulmartinezhorta earlylanguageimpairmentasanintegralpartofthecognitivephenotypeinhuntingtonsdisease |