- ICH GCP
- US Clinical Trials Registry
- Clinical Trial NCT03560661
Acoustic and Perceptual Markers of Dysarthria in Amyotrophic Lateral Sclerosis (ALS) (SPEECH-ALS)
January 27, 2020 updated by: Assistance Publique - Hôpitaux de Paris
Identification of Acoustic and Perceptual Markers of Lower and Upper Motor Neuron Signs in Dysarthria of Patients With Amyotrophic Lateral Sclerosis : a Comparison With Primitive Lateral Sclerosis, Kennedy's Disease and Controls
This study proposes to identify acoustic and perceptual markers related to upper motor neuron (UMN) degeneration and lower motor neuron (LMN) degeneration in the dysarthria of patients with amyotrophic lateral sclerosis (ALS) which involves the degeneration of both systems.
ALS patients will be gathered in clinical groups according to electromyogram (EMG) and clinical signs observed in the bulbar site.
UMN signs are defined as jaw clonus, gag reflex and pseudobulbar features (lability).
LMN signs are defined as lingual atrophy and fasciculations.
The dysarthria will be compared to dysarthria of patients involving an exclusive UMN system degeneration (in primitive lateral sclerosis) and an exclusive LMN system degeneration (Kennedy's disease).
Patients will be compared to the controls who permitted to establish the standards of the "MonPaGe" tool.
MonPaGe is a computerized tool based on a multidimensional and quantified assessment of voice and speech, by a set of targeted acoustic and perceptual criteria.
Study Overview
Status
Completed
Detailed Description
ALS is a motor neuron disease characterized by a progressive degeneration of motor neurons in the brain, brainstem, and spinal cord.
Degeneration of the upper and lower motor neurons (UMN and LMN) leads to spasticity, impaired reflexes, muscle fatigue, muscle weakness and atrophy.
PLS is a motor neuron disease involving exclusively the UMN system and Kennedy's disease is a genetic condition involving the LMN system.
Those three different motor neuron diseases can lead to a dysarthria.
Affected individuals with ALS vary significantly in the locus of disease onset, presentation at diagnosis and rate of progression.
Regardless of the site of onset, most patients with ALS will experience bulbar motor deterioration that will lead to a dysarthria.
According to Darley's classification of dysarthrias, dysarthria in ALS is grossly described as "mixed" (both spastic, due the UMN deterioration, and flaccid, due to the LMN deterioration).
However, at the onset of bulbar signs, when the dysarthria is still mild, dysarthric profiles and bulbar clinical signs can vary within individuals.
Perceptual and acoustic features of dysarthria in ALS have been studied but they have not been studied in dysarthric patients with PLS and KD.
This study was motivated by the need to better understand the dysarthria in ALS and it's management in speech therapy.
The goals of this study is to question the impact of the degeneration of the UMN system versus the LMN system on the speech motor system and see if the investigators can identify acoustic and perceptual markers related to UMN degeneration on one side and LMN degeneration on the other side.
The investigators will compare acoustic and perceptual features between the recorded speech of different clinical groups.
Clinical groups will be made according to clinical signs and EMG.
The clinical signs for UMN involvement in the bulbar region are: gag reflex, jaw clonus, pseudobulbar features (lability).
The clinical signs for LMN involvement are: lingual atrophy and fasciculation.
The population will be composed by groups of ALS-LMN patients, ALS-UMN patients , PLS patients and KD patients and a control group already recruited by the LPP (Laboratoire de Phonétique et de Phonologie) team (CNRS...) which permitted to establish the standards of the "MonPaGe" tool that the investigators will use to analyse patients recorded speech.
This tool is based on a multidimensional and quantified assessment of voice and speech, by a set of targeted acoustic and perceptual criteria.
Study Type
Observational
Enrollment (Actual)
71
Contacts and Locations
This section provides the contact details for those conducting the study, and information on where this study is being conducted.
Study Locations
-
-
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Paris, France, 75013
- APHP - Hôpital Pitié-Salpêtrière
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-
Participation Criteria
Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.
Eligibility Criteria
Ages Eligible for Study
16 years and older (Adult, Older Adult)
Accepts Healthy Volunteers
No
Genders Eligible for Study
All
Sampling Method
Non-Probability Sample
Study Population
We will recruit 80 patients (40 ALS patients, 20 PLS patients and 20 KD patients).
They will be recruited at the "centre de référence pour les maladies du motoneurone (Pitié-Salpêtrière).
An initial EMG will be achieved during the usual diagnostic management
Description
Inclusion Criteria:
- Major patients diagnosed at the "centre référent des maladies du motoneurone" with ALS, PLS or KD,
- French motherhood language
- Mild to moderate severity of dysarthria according to the Perceptual Score of the BECD
- Without respiratory troubles according to the neurologist
- Without dementia.
Exclusion Criteria:
- None
Study Plan
This section provides details of the study plan, including how the study is designed and what the study is measuring.
How is the study designed?
Design Details
- Observational Models: Other
- Time Perspectives: Prospective
Cohorts and Interventions
Group / Cohort |
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Amyotrophic lateral sclerosis (ALS) patients
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Primitive Lateral Sclerosis (PLS) patients
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Kennedy's disease (KD) patients
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What is the study measuring?
Primary Outcome Measures
Outcome Measure |
Measure Description |
Time Frame |
---|---|---|
Number of patients in each patterns of dysarthria
Time Frame: 1 day
|
Patterns of dysarthria will be determined with post hoc unsupervised machine learning method (cluster analysis) from following measurements:
|
1 day
|
Secondary Outcome Measures
Outcome Measure |
Measure Description |
Time Frame |
---|---|---|
Functional scale with the Norris score
Time Frame: 1 day
|
The functional capacities of the bulbar site will be evaluated by the speech therapist with the Norris score.This examination will permit to identify UMN and LMN signs
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1 day
|
Functional scale with the ALS functional rating scale-R (ALS FRS-R)
Time Frame: 1 day
|
The functional capacities of the bulbar site will be evaluated by the speech therapist with the ALS functional rating scale-R (ALS FRS-R).
This examination will permit to identify UMN and LMN signs
|
1 day
|
Severity of the dysarthria
Time Frame: 1 day
|
A severity score of the dysarthria will be calculated with the Perceptual Score of the Batterie d'Evaluation Clinique de la Dysarthrie (BECD)
|
1 day
|
Clinical examination
Time Frame: 1 day
|
Classical speech therapy clinical examination
|
1 day
|
Collaborators and Investigators
This is where you will find people and organizations involved with this study.
Collaborators
Investigators
- Principal Investigator: Nathalie LEVEQUE, Assistance Publique Hoptiaux de Paris
Publications and helpful links
The person responsible for entering information about the study voluntarily provides these publications. These may be about anything related to the study.
Study record dates
These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.
Study Major Dates
Study Start (Actual)
July 5, 2018
Primary Completion (Actual)
December 23, 2019
Study Completion (Actual)
December 23, 2019
Study Registration Dates
First Submitted
January 2, 2018
First Submitted That Met QC Criteria
June 6, 2018
First Posted (Actual)
June 18, 2018
Study Record Updates
Last Update Posted (Actual)
January 29, 2020
Last Update Submitted That Met QC Criteria
January 27, 2020
Last Verified
January 1, 2020
More Information
Terms related to this study
Keywords
Additional Relevant MeSH Terms
- Pathologic Processes
- Metabolic Diseases
- Central Nervous System Diseases
- Nervous System Diseases
- Neurologic Manifestations
- Neurobehavioral Manifestations
- Genetic Diseases, Inborn
- Genetic Diseases, X-Linked
- Neuromuscular Diseases
- Neurodegenerative Diseases
- Spinal Cord Diseases
- TDP-43 Proteinopathies
- Proteostasis Deficiencies
- Heredodegenerative Disorders, Nervous System
- Language Disorders
- Communication Disorders
- Speech Disorders
- Articulation Disorders
- Muscular Atrophy, Spinal
- Sclerosis
- Motor Neuron Disease
- Amyotrophic Lateral Sclerosis
- Bulbo-Spinal Atrophy, X-Linked
- Dysarthria
Other Study ID Numbers
- NI17040J
- 2017-A03151-52 (Registry Identifier: IDRCB)
Drug and device information, study documents
Studies a U.S. FDA-regulated drug product
No
Studies a U.S. FDA-regulated device product
No
This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.
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