Language in Huntington Disease (2014-)

On December 7, 2015 by Antonia Tovar

Lead researchers in the group: Catalina Morey Matamalas (Study 1); Antonia Tovar Torres (Study 2)

Collaborators: Ruth de Diego (Psychology, UB); FIDMAG Germanes Hospitalàries Research Foundation; Dr. Jesús Ruiz (Hospital Mare de Déu de la Mercè; Vitor Zimmerer (UCL); Rosemary Varley (UCL)

Both studies seek to describe in detail the linguistic profile of patients with Huntington’s Disease at different stages of their illness. In HD, problems related to language capacity have been barely studied, and they are often considered a direct consequence of disturbances in the sensory-motor system. The main purpose of the present project is to investigate in which way and to what extent there is change in structural aspects of language that can illuminate cognitive decline seen in this population, both before and after the emergence of motor symptoms. Study 1 focuses on analyzing narrative (story re-telling), Study 2 expands the first study by looking at linguistic comprehension as well, and at correlations between linguistic understanding and measures of ‘theory of mind’.

Results from Study 1

  1.  Patients show deficits related to the Quantitative domain, the Fluency domain, the Sentence Connectivity domain, the Reference domain and the Concordance domain.
  2. In pre-symptomatic gene carriers, before standard tests detect any decline in the cognitive profile, the Sentence Connectivity domain and the Reference domain can be deteriorated.

Results from Study 2

  1. The linguistic profile of patients is characterized by referential problems (vague referents), deterioration of the  discursive cohesion (vague topics and ambivalence) and syntactic simplification (over-use of coordination).
  2.  Language is also affected in pre-symptomatic gene carriers. This linguistic decline is seen in the domains of Fluency and Sentence Connectivity.
  3. Language difficulties relate to difficulties in comprehending ToM-related content especially in a non-linguistic domain, and to grasp the meaning of complex sentences describing mental states (SPM task).
  4. Overall, these results suggest that cognitive decline in HD due to striatal neurodegeneration not only affects language but more specifically the linguistic resources required for representing mental states.

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