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

NCT03560661 | Completed

• 2 other identifiers: NI17040J2017-A03151-52
• Study Type: observational
• Target: 71
• Locations: 1 country
• Sites: 1

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.

Conditions

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

Keywords

Acoustic and perceptual markers; Dysarthria; Amyotrophic lateral sclerosis; Primitive lateral sclerosis; Kennedy's disease

Outcome Measures

Primary Outcomes (1)

• Number of patients in each patterns of dysarthria

  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).

  1 day

Secondary Outcomes (4)

• Functional scale with the Norris score

  1 day

• Functional scale with the ALS functional rating scale-R (ALS FRS-R)

  1 day

• Severity of the dysarthria

  1 day

• Clinical examination

  1 day

Study Arms (3)

Amyotrophic lateral sclerosis (ALS) patients

Primitive Lateral Sclerosis (PLS) patients

Kennedy's disease (KD) patients

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• 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

You may qualify if:

• 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.

You may not qualify if:

• None

Sponsors & Collaborators

• Assistance Publique - Hôpitaux de Paris: lead
• Centre National de la Recherche Scientifique, France: collaborator

Study Sites (1)

APHP - Hôpital Pitié-Salpêtriere

Paris, 75013, France

Location

Related Publications (1)

• 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.

  PMID: 33639779 DERIVED

MeSH Terms

Conditions

Amyotrophic Lateral Sclerosis; Motor Neuron Disease; Bulbo-Spinal Atrophy, X-Linked; Dysarthria

Condition Hierarchy (Ancestors)

Spinal Cord Diseases; Central Nervous System Diseases; Nervous System Diseases; Neurodegenerative Diseases; TDP-43 Proteinopathies; Neuromuscular Diseases; Proteostasis Deficiencies; Metabolic Diseases; Nutritional and Metabolic Diseases; Muscular Atrophy, Spinal; Heredodegenerative Disorders, Nervous System; Genetic Diseases, X-Linked; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Articulation Disorders; Speech Disorders; Language Disorders; Communication Disorders; Neurobehavioral Manifestations; Neurologic Manifestations; Signs and Symptoms; Pathological Conditions, Signs and Symptoms

Study Officials

• Nathalie LEVEQUE

  Assistance Publique Hoptiaux de Paris

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: OTHER
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: January 2, 2018
• First Posted: June 18, 2018
• Study Start: July 5, 2018
• Primary Completion: December 23, 2019
• Study Completion: December 23, 2019
• Last Updated: January 29, 2020

Record last verified: 2020-01

Locations

FR (1)