Objective: To explore the utility of objective analysis of speech as a quantifiable clinical biomarker in premanifest HD.

Background: The autosomal dominant inheritance of Huntington’s Disease (HD) allows measurement of disease progression prior to symptom onset according to diagnostic criteria. Measuring these subtle changes using behavioral testing is challenging. Speech disturbance is a common but under-utilized clinical feature of premanifest HD.

Method: Speech was elicited from 55 individuals with the expanded HD gene (14 presymptomatic individuals, PresymHD; 18 prodromal individuals, ProdromHD; 14 early stage HD; 9 mid stage HD). Age- and sex-matched control subjects for each group were also recruited (n=55). Participants produced syllable repetition tasks, reading, monologue and a sustained vowel. Speech data were analysed objectively using acoustic methodology. The Cogstate Brief Battery and the Purdue Pegboard Test were used to examine cognitive and fine motor performance.

Results: Speech timing differed between PresymHD (furthest group from disease onset) and matched controls on the monologue task only, the most cognitively demanding test in the protocol. There was a significant difference on measures of single and paired syllable duration and repetition rate between ProdromHD (within 15 years to estimated disease onset) and healthy controls. Voice quality did not differ between controls and PresymHD or ProdromHD group. Associations between cognition and fine motor control were explored. Speech appeared to be more sensitive to pre-HD fine motor control than cognitive function.

Conclusion: Speech tasks with predominately motor (eg. syllable repetition) and more cognitively demanding tasks (eg. monologue) were different to controls before diagnosis. The largest differences were observed in tasks with combined cognitive and motor requirements, highlighting challenges experienced by patients in formulating novel language while performing complex motor tasks. Data  suggest that speech is a subtle, quantifiable marker in premanifest HD.

References: Chan JCS, Stout JC, Vogel AP. Speech in prodromal and symptomatic Huntington’s disease as a model of measuring onset and progression in dominantly inherited neurodegenerative diseases. Neurosci Biobehav Rev. 2019;107:450–460. doi:10.1016/j.neubiorev.2019.08.009

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MDS Abstracts - https://www.mdsabstracts.org/abstract/speech-is-a-sensitive-marker-in-premanifest-huntingtons-disease-exploring-acoustic-cognitive-and-fine-motor-performance/