Learn More
UNLABELLED The purpose of this study was to examine the relationships among speech intelligibility and communication effectiveness as rated by speakers and their listeners. Participants completed procedures to measure (a) speech intelligibility, (b) self-perceptions of communication effectiveness, and (c) listener (spouse or family member) perceptions of(More)
Mitochondria are abnormal in persons with amyotrophic lateral sclerosis (ALS) for unknown reasons. We explored whether aberration of mitochondrial DNA (mtDNA) could play a role in this by transferring mitochondrial DNA (mtDNA) from ALS subjects to mtDNA-depleted human neuroblastoma cells. Resulting ALS cytoplasmic hybrids (cybrids) exhibited abnormal(More)
A model of the 40 Hz auditory event-related potential (40 Hz AERP) was developed and evaluated in a group of normal subjects and two patient groups. The model views the 40 Hz AERP as a combination of the brain-stem auditory evoked potential (BAEP) and a sinusoidal component presumed to arise from structures rostral to the brain-stem. Fourier analysis(More)
Oxidative abnormalities have been identified both in familial amyotrophic lateral sclerosis (FALS) and the more prevalent sporadic ALS (SALS). Mitochondria dysfunction and toxic free radicals may play a role in this disease process, although the exact pathogenesis of both forms of ALS remains unknown. 2,3-DHBA is a hydroxylated salicylate by product that(More)
R+ pramipexole (PPX) is a lipophilic cation that concentrates into brain and mitochondria and efficiently scavenges reactive oxygen and nitrogen species (RONS). Under the auspices of a Physician-Sponsor IND, R+PPX was dosed to small numbers of ALS patients for tolerability and safety while efficacy measures were also collected. The purpose of this paper is(More)
UNLABELLED The goal of this study was to investigate the deterioration of lip and jaw movements during speech longitudinally in three individuals diagnosed with bulbar amyotrophic lateral sclerosis (ALS). The study was motivated by the need to understand the relationship between physiologic changes in speech movements and clinical measures of speech(More)
Bulbar motor deterioration due to amyotrophic lateral sclerosis (ALS) leads to the eventual impairment of speech and swallowing functions. Despite these devastating consequences, no standardized diagnostic procedure for assessing bulbar dysfunction in ALS exists and adequate objective markers of bulbar deterioration have not been identified. In this paper,(More)
This study investigated speaking rate effects on articulatory pattern consistency in talkers with mild amyotrophic lateral sclerosis (ALS) to better understand speech rate declines during the early stages of speech deterioration. Eight talkers with mild ALS and 11 controls repeated a sentence at their typical rate, an accelerated rate, and a reduced rate.(More)
Improved methods for assessing bulbar impairment are necessary for expediting diagnosis of bulbar dysfunction in ALS, for predicting disease progression across speech subsystems, and for addressing the critical need for sensitive outcome measures for ongoing experimental treatment trials. To address this need, we are obtaining longitudinal profiles of(More)
PURPOSE The goal of this study was to determine if talkers with ALS are limited in their ability to increase lower lip and jaw speed at an early stage of the disease when their speaking rate and intelligibility are only minimally or not affected. METHOD A novel metronome paced fixed-target task was used to assess movement speed capacities during lower lip(More)