Characterization and Quantification of Motor Speech Disorders in Huntington's Disease: Identification of Acoustic Markers

Huntington's disease (HD) is a neurodegenerative disorder of the central nervous system, of genetic etiology. It manifests itself in motor, cognitive and psychiatric disorders, which progressively worsen until the patient is grabbed. Motor speech performance disorders are present from the early stage of the disease. These difficulties, which progressively worsen until the end of the patient's life, lead to a communication disorder and constitute a major handicap with social withdrawal, loss of self-esteem and isolation, which are always very painfully experienced by patients and their entourage. Dysarthria in the HD has been very little studied, which makes its speech therapy uncodified. According to the most widely used classification by speech therapists and neurologists, the Darley classification (1975), the speech disorders observed in the HD are included in the heterogeneous group of hyperkinetic dysarthria, whose contours and properties are ill-defined. In this context, a more precise knowledge of speech disorders in the HD appears necessary to identify and characterize the different types of dysarthria and the dysfunctions associated with each level of production (respiration, phonation, resonance, articulation, prosody) and thus improve speech language therapy.

The investigators propose to use an acoustic evaluation to support the perceptual evaluation, available in the MonPaGe protocol, to describe the fine characteristics of the speech disorders in the HD and to define objective markers of dysarthria in this disease for francophone patients.

Each patient is seen in the framework of his annual follow-up consultation in the Competence Center mdH of Bordeaux University Hospital or in the Reference Center mdH of the hospital Henri Mondor of Créteil, for an evaluation of his speech with the computerized tool MonPaGe. A first speech evaluation with the BECD (Auzou, 2006) during 30 minutes is proposed to exclude very severe dysarthria. At the end of this rapid test subtest, the subjects who obtain a perceptive score <16 are offered the complete assesment of the MonPaGe computerized protocol (30 minutes).The application records directly and anonymously stores speech productions made by the patient.The productions are transmitted to the Phonetics and Phonology Laboratory of Paris (UMR 7018 CNRS / Sorbonne Nouvelle), via a secure large data exchange server and are subject to a perceptual and acoustic evaluation a posteriori.

Inclusion criteria

• major patients
• francophone patients
• Huntington's disease carriers confirmed by molecular biological analysis
• carriers of pre-symptomatic Huntington's disease: Total Motor Score of the Unified Huntington's Disease Rating Scale <5
• carriers of Huntington's disease at a declared stage: Total Motor Score of the Unified Huntington's Disease Rating Scale ≥ 5
• followed in the Competence Center Huntington's disease Bordeaux Hospital or in the Reference Center Huntington's disease Henri Mondor Hospital of Créteil
• affiliates or beneficiaries of a social security scheme
• Finally, a free, informed and express consent of the patient must be collected.

Exclusion criteria

• Huntington's disease patients with severe dysarthria (BECD score> 16), acoustic analysis not being possible on unintelligible speech
• patients placed under the protection of justice