Characterization of the Profile of Dysarthria in Huntington's Disease, Using the Clinical Evaluation Battery of Dysarthria (Huntington)

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.

Study Overview

• Status: Withdrawn
• Conditions: Huntington Disease; Dysarthria
• Intervention / Treatment: Other: Study of dysarthria in patients with Huntington's disease

• Study Type: Interventional
• Phase: Not Applicable

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

Description

Inclusion Criteria:

• Diagnosed Patients HD
• Major Patients
• French-speaking patients
• MMS score greater than 20
• Patients with informed consent signed
• Patients covered by a social insurance system

Exclusion Criteria:

• Patients with intercurrent pathologies or other psychiatric disorders that may interfere with battery replacement (at the discretion of the investigator)
• Illiteracy
• Persons placed under safeguard of justice (adults under guardianship or guardianship) or deprived of their liberty

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Other
• Allocation: N/A
• Interventional Model: Single Group Assignment
• Masking: None (Open Label)

Number of Arms

1

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
Other: Patient with Huntington's Disease	Other: Study of dysarthria in patients with Huntington's disease; Study of dysarthria in patients with Huntington's disease

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Time Frame
Analysis of the Clinical Evaluation Battery of Dysarthria using criteria to establish a clinical profile specific to Huntington's disease	1 day

Collaborators and Investigators

• Sponsor: Centre Hospitalier Universitaire, Amiens

Study record dates

Study Major Dates

• Study Start (Actual): March 26, 2015
• Primary Completion (Actual): March 1, 2017
• Study Completion (Actual): March 1, 2017

Study Registration Dates

• First Submitted: May 3, 2017
• First Submitted That Met QC Criteria: May 3, 2017
• First Posted (Actual): May 5, 2017

Study Record Updates

• Last Update Posted (Actual): May 5, 2017
• Last Update Submitted That Met QC Criteria: May 3, 2017
• Last Verified: May 1, 2017

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Mental Disorders; Brain Diseases; Central Nervous System Diseases; Nervous System Diseases; Neurologic Manifestations; Neurobehavioral Manifestations; Neurocognitive Disorders; Genetic Diseases, Inborn; Basal Ganglia Diseases; Movement Disorders; Neurodegenerative Diseases; Dyskinesias; Heredodegenerative Disorders, Nervous System; Dementia; Cognition Disorders; Language Disorders; Communication Disorders; Speech Disorders; Chorea; Articulation Disorders; Huntington Disease; Dysarthria
• Other Study ID Numbers: PI2015_843_0005

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No