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Acoustic and Perceptual Markers of Dysarthria in Amyotrophic Lateral Sclerosis (ALS) (SPEECH-ALS)

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ClinicalTrials.gov Identifier: NCT03560661
Recruitment Status : Recruiting
First Posted : June 18, 2018
Last Update Posted : August 14, 2018
Sponsor:
Collaborator:
Centre National de la Recherche Scientifique, France
Information provided by (Responsible Party):
Assistance Publique - Hôpitaux de Paris

Brief Summary:
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.

Condition or disease
Amyotrophic Lateral Sclerosis Primary Lateral Sclerosis Kennedy's Disease

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.

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Study Type : Observational
Estimated Enrollment : 80 participants
Observational Model: Other
Time Perspective: Prospective
Official Title: 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
Actual Study Start Date : July 5, 2018
Estimated Primary Completion Date : April 2019
Estimated Study Completion Date : April 2019


Group/Cohort
Amyotrophic lateral sclerosis (ALS) patients
Primitive Lateral Sclerosis (PLS) patients
Kennedy's disease (KD) patients



Primary Outcome Measures :
  1. 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:

    • Intelligibility: number of words recognized (score 0 to 15)
    • Maximum phonation time (TMP), (milliseconds),
    • Phonation: mean and standard deviation of fundamental frequency (Hz); jitter (%), shimmer (%);Signal to Noise Ratio (dB)
    • Intensity modulation capacities (score 0 to 4),
    • Articulation of consonants and vowels : global error score (score 0 to 53); qualitative profile of error; acoustic range variation (Hz),
    • Coarticulation (Hz),
    • Score of prosody,
    • Verbal Diadococinesis : accuracy (score 0 to 4) ; control (score 0 to 4) and flow rate (syllables/sec),
    • Speech flow rate (syllables/sec).


Secondary Outcome Measures :
  1. 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

  2. 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

  3. 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)

  4. Clinical examination [ Time Frame: 1 day ]
    Classical speech therapy clinical examination



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Ages Eligible for Study:   18 Years and older   (Adult, Older Adult)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   No
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
Criteria

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:


Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT03560661


Contacts
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Contact: Nathalie LEVEQUE +331 42 16 15 95 nathalie.leveque@aphp.fr

Locations
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France
APHP - Hôpital Pitié-Salpêtriere Recruiting
Paris, France, 75013
Contact: Nathalie LEVEQUE    33(0)1 42 16 15 95    nathalie.leveque@aphp.fr   
Sponsors and Collaborators
Assistance Publique - Hôpitaux de Paris
Centre National de la Recherche Scientifique, France
Investigators
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Principal Investigator: Nathalie LEVEQUE Assistance Publique Hoptiaux de Paris

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Responsible Party: Assistance Publique - Hôpitaux de Paris
ClinicalTrials.gov Identifier: NCT03560661     History of Changes
Other Study ID Numbers: NI17040J
2017-A03151-52 ( Registry Identifier: IDRCB )
First Posted: June 18, 2018    Key Record Dates
Last Update Posted: August 14, 2018
Last Verified: April 2018

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Studies a U.S. FDA-regulated Drug Product: No
Studies a U.S. FDA-regulated Device Product: No

Keywords provided by Assistance Publique - Hôpitaux de Paris:
Acoustic and perceptual markers
Dysarthria
Amyotrophic lateral sclerosis
Primitive lateral sclerosis
Kennedy's disease

Additional relevant MeSH terms:
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Sclerosis
Motor Neuron Disease
Amyotrophic Lateral Sclerosis
Dysarthria
Bulbo-Spinal Atrophy, X-Linked
Pathologic Processes
Neurodegenerative Diseases
Nervous System Diseases
Neuromuscular Diseases
Spinal Cord Diseases
Central Nervous System Diseases
TDP-43 Proteinopathies
Proteostasis Deficiencies
Metabolic Diseases
Articulation Disorders
Speech Disorders
Language Disorders
Communication Disorders
Neurobehavioral Manifestations
Neurologic Manifestations
Signs and Symptoms
Muscular Atrophy, Spinal
Heredodegenerative Disorders, Nervous System
Genetic Diseases, X-Linked
Genetic Diseases, Inborn