Speech biomarkers in Huntington's disease: A cross-sectional study in pre-symptomatic, prodromal and early manifest stages.

Kouba, Tomas; Frank, Wiebke; Tykalova, Tereza; Mühlbäck, Alzbeta; Klempíř, Jiří; Lindenberg, Katrin S; Landwehrmeyer, G Bernhard; Rusz, Jan (2023). Speech biomarkers in Huntington's disease: A cross-sectional study in pre-symptomatic, prodromal and early manifest stages. European journal of neurology, 30(5), pp. 1262-1271. Wiley 10.1111/ene.15726

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BACKGROUND

Motor speech alterations are a prominent feature of clinically manifest Huntington disease (HD). Objective acoustic analysis of speech can quantify speech alterations. It is currently unknown, however, at what stage of HD speech alterations can be reliably detected.

AIM

We explored patterns and extent of speech alterations using objective acoustic analysis in HD and explored correlations to rater-assessed phenotypical features as well to biological determinants of HD.

METHODS

Speech samples were acquired from 44 premanifest (29 pre-symptomatic and 15 prodromal) and 25 manifest HD gene expansion carriers, and 25 matched healthy controls. A quantitative automated acoustic analysis of 10 speech dimensions was performed.

RESULTS

Automated speech analysis allowed to differentiate between HD and controls with an area under the curve of 0.74 for pre-symptomatic, 0.92 for prodromal, and 0.97 for manifest stages. In addition to irregular alternating motion rates and prolonged pauses seen only in manifest HD, both prodromal and manifest HD displayed slowed articulation rate, slowed alternating motion rates, increased loudness variability, and unstable steady state position of articulators. In premanifest subjects, speech alteration severity was associated with cognitive slowing (r=-0.52, p<0.001) and the extent of bradykinesia (r=0.43, p=0.004). Speech alterations correlated with a measure of exposure to mutant gene products (CAP scores; r=0.60, p<0.001).

CONCLUSION

Speech abnormalities in HD are associated with other motor and cognitive deficits and are measurable already in premanifest stages of HD. Therefore, automated speech analysis might represent a quantitative HD biomarker with potential for assessing disease progression.

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5 Citations in Web of Science ®
6 Citations in Scopus

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