Motor speech patterns in Huntington disease

Abstract

ObjectiveDysarthric speech of persons with Huntington disease (HD) is typically described as hyperkinetic; however, studies suggest that dysarthria can vary and resemble patterns in other neurologic conditions. To test the hypothesis that distinct motor speech subgroups can be identified within a larger cohort of patients with HD, we performed a cluster analysis on speech perceptual characteristics of patient audio recordings.MethodsAudio recordings of 48 patients with mild to moderate dysarthria due to HD were presented to 6 trained raters. Raters provided scores for various speech features (e.g., voice, articulation, prosody) of audio recordings using the classic Mayo Clinic dysarthria rating scale. Scores were submitted to an unsupervised k-means cluster analysis to determine the most salient speech features of subgroups based on motor speech patterns.ResultsFour unique subgroups emerged from the cohort of patients with HD. Subgroup 1 was characterized by an abnormally fast speaking rate among other unique speech features, whereas subgroups 2 and 3 were defined by an abnormally slow speaking rate. Salient speech features for subgroup 2 overlapped with subgroup 3; however, the severity of dysarthria differed. Subgroup 4 was characterized by mild deviations of speech features with typical speech rate. Length of CAG repeats, Unified Huntington’s Disease Rating Scale total motor score, and percent intelligibility were significantly different for pairwise comparisons of subgroups.ConclusionThis study supports the existence of distinct presentations of dysarthria in patients with HD, which may be due to divergent pathologic processes. The findings are discussed in relation to previous literature and clinical implications.

Classification of Manifest Huntington Disease Using Vowel Distortion Measures

“…FVDM provide information that is supplementary to existing speech features, and improve manifest HD classification accuracy from 63% to 80%. Furthermore, we observe that the relationship between FVDM and HD manifestation is consistent with understandings of vowel distortion within HD [11][12][13].…”

“…Vowel distortion is a prevalent symptom of HD [11][12][13]. Prior works have quantified vowel distortion from sustained vowels using a variety of perturbation measures, and used these measures to classify a range of disorders.…”

Vocal Markers from Sustained Phonation in Huntington’s Disease

“…Motor speech disorders in Huntington's Disease are commonly referred to as hyperkinetic dysarthria [8]: variable rate, abnormal prosody, imprecise consonants and distorted vowels, phonation deviations, and sudden forced breath. Yet, there is some recent evidence that the speech motor impairments in Huntington's Disease are highly heterogeneous [9].…”

Striatum and language processing: Where do we stand?

“…In HD, the deficit of the inhibitory system leads to involuntary choreatic movements, psychomotor slowing, and reduced agility of eye movements and articulation (Snowden, 2017). Dysarthria is usually the first symptom reported in these patients (Diehl et al, 2019;Podoll, Caspary, Lange, & Noth, 1988). Speech rate correlates with motor impairment as, for example, tapping ability in a quantitative motor assessment (Skodda et al, 2014(Skodda et al, , 2016.…”