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

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.

Reference Across Pathologies: A New Linguistic Lens on Disorders of Thought

“…Human beings engage in acts of reference of a kind not seen in other species, using grammar and words as an inherent aspect of their peculiar mode of thought. Evidence reviewed here suggests a dysfunction of language at the level of its referential function in both ASD and SZ (for evidence for the same conclusion in Huntington's disease, see Hinzen et al 2017). Critical reflection has to be devoted to the prevailing assumption that clinical language dysfunction needs to be either language-specific or else be the secondary consequence of a primary 'cognitive' impairment, a disturbance of 'thought'.…”

“…The language-thought dichotomy (for this particular type of thought) thus collapses, thereby erasing a standard chicken-and-egg problem as well. This latter view will here be called 'un-Cartesian' and be developed in Section 2 as it has evolved over the last decade (via Hinzen 2007;Sheehan and Hinzen 2011;Arsenijevic and Hinzen 2012;Hinzen 2012Hinzen , 2013Hinzen , 2014aHinzen , 2014bHinzen , 2015Hinzen and Sheehan 2013;Martin and Hinzen 2014;Hinzen and Schroeder 2015;Mattos and Hinzen 2015;Hinzen and Rossello 2015;Hinzen et al 2016b;Hinzen et al 2017). 3 Both Cartesian and un-Cartesian views of language can be empirically explored in the context of cognitive disorders, which is the subject of Section 3.…”

Deficits in nominal reference identify thought disordered speech in a narrative production task

“…Such a profile can contribute to the cognitive neuropsychology of TD. Moreover, across different cognitive disorders, the potential role of language as a clinical marker has been emphasized based on evidence for its role in prediction, diagnosis, and tracking the disease process involved, including in schizophrenia [ 3 – 9 ], autism spectrum disorders [ 10 ], depression [ 11 ], Huntington’s disease [ 12 ], and Alzheimer’s [ 13 ].…”

Similitudes y diferencias en el déficit lingüístico de dos casos de huntington con variables médicas similares

“…En primer lugar, y siendo el artículo más relacionado con el tema de este proyecto, aludimos el estudio «A systematic linguistic profile of spontaneous narrative speech in pre-sintomatic and early stage Huntington's disease» (Hinzen et al, 2018). Como mencionamos en la introducción de este trabajo, se trata de un estudio que sigue una metodología parecida a la que utilizamos aquí; no obstante, se evalúan muchos menos aspectos.…”