Huntington Disease Clinical Trial
Official title:
Characterization of the Profile of Dysarthria in Huntington's Disease, Using the Clinical Evaluation Battery of Dysarthria
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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