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