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

NCT ID: NCT03560661

Last Updated: 2020-01-29

Basic Information

• Recruitment Status: COMPLETED
• Total Enrollment: 71 participants
• Study Classification: OBSERVATIONAL
• Study Start Date: 2018-07-05
• Study Completion Date: 2019-12-23

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.

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.

Conditions

Amyotrophic Lateral Sclerosis; Primary Lateral Sclerosis; Kennedy's Disease

Study Design

• Observational Model Type: OTHER
• Study Time Perspective: PROSPECTIVE

Study Groups

Amyotrophic lateral sclerosis (ALS) patients

No interventions assigned to this group

Primitive Lateral Sclerosis (PLS) patients

No interventions assigned to this group

Kennedy's disease (KD) patients

No interventions assigned to this group

Eligibility 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

• None

• Minimum Eligible Age: 18 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

Centre National de la Recherche Scientifique, France

OTHER

Sponsor Role: collaborator

Assistance Publique - Hôpitaux de Paris

OTHER

Sponsor Role: lead

Responsible Party

Responsibility Role: SPONSOR

Principal Investigators

Nathalie LEVEQUE

Role: PRINCIPAL_INVESTIGATOR

Assistance Publique Hoptiaux de Paris

Locations

APHP - Hôpital Pitié-Salpêtriere

Paris, , France

Countries

France

References

Slis A, Leveque N, Fougeron C, Pernon M, Assal F, Lancia L. Analysing spectral changes over time to identify articulatory impairments in dysarthria. J Acoust Soc Am. 2021 Feb;149(2):758. doi: 10.1121/10.0003332.

Reference Type: DERIVED

PMID: 33639779 (View on PubMed)

Other Identifiers

2017-A03151-52

Identifier Type: REGISTRY

Identifier Source: secondary_id

NI17040J

Identifier Type: -

Identifier Source: org_study_id