The diagnostic utility of patient-report and speech-language pathologists’ ratings for detecting the early onset of bulbar symptoms due to ALS

Abstract

Objective: This study aimed to determine the diagnostic utility of clinician speech ratings and patient self-report for detecting early bulbar changes associated with amyotrophic lateral sclerosis (ALS), compared to instrumentation-based speech measures.

Methods: Thirty-six individuals with ALS and 17 healthy control participants were included. Patients’ awareness of early bulbar motor involvement was assessed using self-reported scores on the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R). Clinicians’ detection of early bulbar motor involvement was assessed through perceptual speech ratings by two experienced speech-language pathologists. Participants with ALS were grouped as ‘bulbar pre-symptomatic’ or ‘bulbar symptomatic’ based on self-report and clinician ratings, and compared to healthy controls on six instrumentation-based speech measures. ROC analysis was used to compare the sensitivity and specificity of perceptual and instrumentation-based measures for detecting bulbar changes in pre-symptomatic individuals.

Results: Early bulbar changes that were documented using instrumentation-based measures were undetected by both patients and clinicians. ROC analyses indicated that instrumentation-based measures outperformed clinicians’ scaled severity ratings, and that percent pause time was the best measure for differentiating healthy controls from bulbar pre-symptomatic individuals with ALS.

Conclusions: Findings suggested that instrumentation-based measures of speech may be necessary for early detection of bulbar changes due to ALS.

Keywords:

• Bulbar
• early detection
• speech motor impairment

Acknowledgments

The authors would like to thank the patients who participated in this study and their families. They also thank Brian Richburg, Meg Simione, and Bridget Perry for their assistance with data collection and analysis.

Declaration of interest

The authors report no conflicts of interest. This research was funded by NIH-NIDCD grants R01DC009890 and R01DC013547.