Clinical trial NCT04630574: Characterization and Quantification of Motor Speech Disorders in Huntington's Disease: Identification of Acoustic Markers
Official title: Characterization and Quantification of Motor Speech Disorders in Huntington's Disease: Identification of Acoustic Markers
Study type: OBSERVATIONAL
Overall status: COMPLETED
Conditions: Huntington Disease
Keywords: Huntington's disease
Dysarthria
Motor speech performance
Acoustic markers
Lead sponsor: University Hospital, Bordeaux
Sponsor class: OTHER
Collaborators: name: Centre National de la Recherche Scientifique, France
First posted: 2020-11-16
Enrollment: 150
Sex: ALL
Minimum age (years): 18
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: CASE_ONLY
Time perspective: PROSPECTIVE
Sampling method: NON_PROBABILITY_SAMPLE
Study population: patients with Huntington's disease at presymptomatic and declared stages of the disease.

Brief summary: The study proposes to identify deviant speech dimensions in patients with HD at presymptomatic and declared stages of the disease, compared to healthy subjects, using the computerized MonPaGe speech protocol. This tool is based on a multidimensional and quantified assessment of voice and speech, by a set of targeted acoustic and perceptual criteria.

Detailed description: 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.

Eligibility criteria: 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

Primary outcomes:
measure: Determination of the intelligibility score.
timeFrame: Day 1
measure: Determination of the maximum phonation time.
timeFrame: Day 1
measure: Determination of the phonation characteristics.
timeFrame: Day 1
measure: Determination of the intensity modulation capacities.
timeFrame: Day 1
measure: Determination of the Coarticulation.
timeFrame: Day 1
measure: Determination of the prosody.
timeFrame: Day 1
measure: Determination of the diadocokinesies,
timeFrame: Day 1
measure: Determination of the articulation rate.
timeFrame: Day 1
Secondary outcomes:
measure: Determination of the Huntington's disease stage.
timeFrame: Day 1
measure: Determination of the clinical motor forms of the Huntington's disease.
timeFrame: Day 1
measure: Determination of the level of cognitive impairment
timeFrame: Day 1
Other outcomes:
Locations:
city: Créteil
country: France
facility: Hôpital Henri-Mondor
city: Talence
country: France
facility: Centre Hospitalier Universitaire de Bordeaux
Study officials:
References: