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A systematic linguistic profile of spontaneous narrative speech in pre-symptomatic and early stage Huntington's disease

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dc.contributor.author Hinzen, Wolfram
dc.contributor.author Rosselló Ximenes, Joana
dc.contributor.author Morey, Cati
dc.contributor.author Camara, Estela
dc.contributor.author Garcia-Gorro, Clara
dc.contributor.author Salvador, Raymond
dc.contributor.author de Diego-Balaguer, Ruth
dc.date.accessioned 2019-10-21T08:47:51Z
dc.date.available 2019-10-21T08:47:51Z
dc.date.issued 2018
dc.identifier.citation Hinzen W, Rosselló J, Morey C, Camara E, Garcia-Gorroe C, Salvador R, Diego-Balaguera Rd. A systematic linguistic profile of spontaneous narrative speech in pre-symptomatic and early stage Huntington's disease. Cortex. 2018; 100:71-83. DOI: 10.1016/j.cortex.2017.07.022.
dc.identifier.issn 0010-9452
dc.identifier.uri http://hdl.handle.net/10230/42470
dc.description.abstract Cognitive decline accompanying the clinically more salient motor symptoms of Huntington's disease (HD) has been widely noted and can precede motor symptoms onset. Less clear is how such decline bears on language functions in everyday life, though a small number of experimental studies have revealed difficulties with the application of rule-based aspects of language in early stages of the disease. Here we aimed to determine whether there is a systematic linguistic profile that characterizes spontaneous narrative speech in both pre-manifest and/or early manifest HD, and how it is related to striatal degeneration and neuropsychological profiles. Twenty-eight early-stage patients (19 manifest and 9 gene-carriers in the pre-manifest stage), matched with 28 controls, participated in a story-telling task. Speech was blindly scored by independent raters according to fine-grained linguistic variables distributed over 5 domains for which composite scores were computed (Quantitative, Fluency, Reference, Connectivity, and Concordance). Voxel-based morphometry (VBM) was used to link specific brain degeneration patterns to loci of linguistic decline. In all of these domains, significant differences were observed between groups. Deficits in Reference and Connectivity were seen in the pre-manifest stage, where no other neuropsychological impairment was detected. Among HD patients, there was a significant positive correlation only between the values in the Quantitative domain and gray matter volume bilaterally in the putamen and pallidum. These results fill the gap of qualitative data of spontaneous narrative speech in HD and reveal that HD is characterized by systematic linguistic impairments leading to dysfluencies and disorganization in core domains of grammatical organization. This includes the referential use of noun phrases and the embedding of clauses, which mediate crucial dimensions of meaning in language in its normal social use. Moreover, such impairment is seen prior to motor symptoms onset and when standardized neuropsychological test profiles are otherwise normal.
dc.format.mimetype application/pdf
dc.language.iso eng
dc.publisher Elsevier
dc.relation.ispartof Cortex. 2018; 100:71-83. DOI: 10.1016/j.cortex.2017.07.022.
dc.rights Publisher under a Creative Commons license
dc.rights.uri https://creativecommons.org/licenses/by-nc-nd/4.0/
dc.title A systematic linguistic profile of spontaneous narrative speech in pre-symptomatic and early stage Huntington's disease
dc.type info:eu-repo/semantics/article
dc.identifier.doi http://dx.doi.org/10.1016/j.cortex.2017.07.022
dc.subject.keyword Huntington's disease
dc.subject.keyword Narrative speech
dc.subject.keyword Grammatical deficits
dc.subject.keyword Voxel-based morphometry
dc.subject.keyword Basal ganglia
dc.rights.accessRights info:eu-repo/semantics/openAccess
dc.type.version info:eu-repo/semantics/publishedVersion

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