Emotion expression through spoken language in Huntington disease

Patients with Huntington's disease suffer from disturbances in the perception of emotions; they do not correctly read the body, vocal and facial expressions of others. With regard to the expression of emotions, it has been shown that they are impaired in expressing emotions through face but up until now, little research has been conducted about their ability to express emotions through spoken language. To better understand emotion production in both voice and language in Huntington's Disease (HD), we tested 115 individuals: 68 patients (HD), 22 participants carrying the mutant HD gene without any motor symptoms (pre-manifest HD), and 25 controls in a single-centre prospective observational follow-up study. Participants were recorded in interviews in which they were asked to recall sad, angry, happy, and neutral stories. Emotion expression through voice and language was investigated by comparing the identifiability of emotions expressed by controls, preHD and HD patients in these interviews. To assess separately vocal and linguistic expression of emotions in a blind design, we used machine learning models instead of a human jury performing a forced-choice recognition test. Results from this study showed that patients with HD had difficulty expressing emotions through both voice and language compared to preHD participants and controls, who behaved similarly and above chance. In addition, we did not find any differences in expression of emotions between preHD and healthy controls. We further validated our newly proposed methodology with a human jury on the speech produced by the controls. These results are consistent with the hypothesis that emotional deficits in HD are caused by impaired sensori-motor representations of emotions, in line with embodied cognition theories. This study also shows how machine learning models can be leveraged to assess emotion expression in a blind and reproducible way.