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Huntington's disease is a hereditary disease of rare autosomal dominant transmission, both neurodegenerative and neuro-psychiatric. Clinically, there are motor symptoms (chorea), cognitive disorders (dementia) and psychiatric disorders.
Among motor disorders, dysarthria is a commonly found symptom. This is classically referred to as hyperkinetic dysarthria according to the criteria of Darley's classification. However, this old classification (1969) is only based on perceptual analysis and lack of specificity.
Moreover, in the course of the disease, chorea (control of the striatal attack D2) decreases to give place to a parkinsonian syndrome (control of the striatal attack D1) and the dysarthria also evolves towards a hypokinetic form . It also seems likely that cerebellar involvement (responsible for ataxia) contributes to dysarthria.
No studies have been published to date to characterize dysarthria in Huntington's disease in a quantified, objective and specific manner. However, Canan Ozsancak describes choreic dysarthria as heterogeneous according to the patients and variable according to the productions. A perceptual study reports an imprecision of the consonants, a lengthening of the pauses, a variable flow, an absence of modulation of the pitch and a hoarse voice.
Finally, few patients are cared for in speech therapy and there is no specific rehabilitation strategy: this would require - and justify a more precise study of the dysarthria of these patients.
The Clinical Evaluation of Dysarthria developed by Pascal Auzou and Véronique Rolland-Monnoury is a recent and partially standardized tool, combining qualitative and quantitative evaluation, which seems adapted to try to better characterize the dysarthria in Huntington's disease.
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