Multiple Orofacial Indices in Amyotrophic Lateral Sclerosis Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by significant speech and swallowing problems resulting from upper and lower motor neuron loss. Weakness is the primary ALS disease-related sign, and measures of muscle strength have revealed nonuniform patterns of muscle weakness in orofacial muscles. To a large extent, ... Research Article
Research Article  |   December 01, 1993
Multiple Orofacial Indices in Amyotrophic Lateral Sclerosis
 
Author Affiliations & Notes
  • Roxanne DePaul
    University of Wisconsin-Whitewater and University of Wisconsin-Madison
  • Benjamin R. Brooks
    University of Wisconsin-Madison and Neurology Service Wm. Middleton VA Hospital
  • Contact author: Roxanne DePaul, Ph.D., University of Wisconsin-Madison, Waisman Center, Rm. 579,1500 Highland Avenue, Madison, Wl 53705-2280.
Article Information
Speech, Voice & Prosodic Disorders / Special Populations / Speech / Research Articles
Research Article   |   December 01, 1993
Multiple Orofacial Indices in Amyotrophic Lateral Sclerosis
Journal of Speech, Language, and Hearing Research, December 1993, Vol. 36, 1158-1167. doi:10.1044/jshr.3606.1158
History: Received March 2, 1993 , Accepted June 15, 1993
 
Journal of Speech, Language, and Hearing Research, December 1993, Vol. 36, 1158-1167. doi:10.1044/jshr.3606.1158
History: Received March 2, 1993; Accepted June 15, 1993

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by significant speech and swallowing problems resulting from upper and lower motor neuron loss. Weakness is the primary ALS disease-related sign, and measures of muscle strength have revealed nonuniform patterns of muscle weakness in orofacial muscles. To a large extent, muscle strength measures in these studies have not been evaluated in terms of functional significance, and few researchers have addressed the relation between weakness and motor neuron loss. This study addressed whether multiple measures, including static isometric maximum voluntary contraction (MVC), a dynamic measure of the peak rate of change of force (PRCF), an upper motor neuron (UMN) index, and a functional disability score (FDS) might enhance understanding of speech dysfunction in ALS. Ten males diagnosed with sporadic ALS showing mild speech impairment and an equal number of matched controls were studied. Tongue MVC and PRCF were more impaired than those of the lip and jaw, irrespective of the time post onset and site of initial symptoms. Results also suggested that disproportionate tongue impairment may be related to UMN deficits. However, impairments in the rate of contraction did not appear to be related to UMN deficits. Tongue weakness and tongue and lower lip PRCF were related to the degree of speech severity, but none of the measures was related to speech intelligibility. The value of a functional outcome measure like speech intelligibility and its role in characterizing orofacial involvement in the early stages of ALS bulbar impairment are discussed.

Acknowledgments
This work was supported in part by NIH grant R29DC00921-01 and the MDA Midwest Regional ALS Research Program grant. The authors would like to thank James H. Abbs, Chris Caldwell, Carl A. Johnson, Raymond Kent, Doris Kistler, Richard Konopacki, Jennifer Parnell, Jean Schumacher, and Melissa Wang for suggestions and/or technical assistance.
Order a Subscription
Pay Per View
Entire Journal of Speech, Language, and Hearing Research content & archive
24-hour access
This Article
24-hour access