Speech profile in patients with Huntington's Disease: cognitive, clinical, and sociodemographic correlations.

Abstract

Purpose: To describe speech profiles in individuals with Huntington's Disease (HD), correlate them with cognitive and clinical aspects, and compare them with healthy controls.

Methods: Symptomatic individuals with a clinical and molecular diagnosis of HD were included. Seven healthy controls, matched by age and sex, were also included. Clinical and sociodemographic data were obtained from medical records. The Unified Huntington's Disease Rating Scale was used to measure severity. Cognitive data were collected using verbal fluency, symbol digit modalities, and Stroop tests. Auditory perceptual assessments were used to evaluate speech, and acoustic analysis extracted information about the following tasks: sustained vowel /a/, utterances with different intonations, oral diadochokinesis, spontaneous speech, and the repeated diphthong /ju:/.

Results: Of the seven individuals with HD, four women with a mean age of 48.86 (±16.03), presented severe (57.15%), moderate (28.57%), and mild (14.28%) dysarthria. Speech impairment in HD case subjects was related to overall motor decline; the worse the motor symptoms, the worse the speech impairment. There was no correlation with the other clinical data or cognition. The case subjects were significantly worse than the control group, specifically regarding the subsystems of phonation (fundamental frequency, phonation time, local jitter, local shimmer), respiration (maximum phonation time) and articulation (speech rate, phonation time in spontaneous speech, number of syllables in spontaneous speech, average duration of syllables and duration of spontaneous speech).

Conclusion: In HD subjects, the most affected speech subsystems were articulation, phonation, and respiration. Poor motor speech patterns were associated with overall motor decline. Speech assessments may provide biomarkers that predict HD progression.