Innovative Measures of Speech and Swallowing Dysfunction in Neurological Disorders (QUESST)

Quantitative Ultrasonography and Electrical Impedance Myography: Speech and Swallowing Technologies for Use in Neurological Disorders

This study is evaluating the use of two painless, non-invasive technologies in the assessment of muscle health over time in both healthy volunteers and patients who have diseases that affect the nervous system.

Study Overview

• Status: Completed
• Conditions: Stroke; Parkinson's Disease; Myasthenia Gravis; Amyotrophic Lateral Sclerosis (ALS); Myopathies

Detailed Description

Speech and swallowing abnormalities are important symptoms associated with disorders of the central nervous system, motor neuron disease (such as amyotrophic lateral sclerosis), myasthenia gravis, and primary muscle conditions. In addition to characterizing the evolution in muscle architecture that could underlie associated orofacial weakness, identifying new ways to measure these abnormalities is critical to the development and testing of novel treatment approaches. As painless, non-invasive, portable technologies, quantitative ultrasonography (QUS) and electrical impedance myography (EIM) could meet the need for objective measures of speech and swallowing dysfunction.

In QUS, acoustic energy is applied to a muscle of interest; the resultant pictorial data are translated into a single value that reflects the health of the imaged muscle. Similarly, in EIM, a high-frequency, low-intensity alternating electrical current is applied to individual muscles, and the resulting voltages measured. Impedance values reflect changes in muscle architecture, including fiber atrophy, inflammation, and the replacement of muscle with fat or connective tissue. Both of these user-friendly methods can provide sensitive indicators of neuromuscular disease status when applied to the limbs. Although they have also been used to evaluate orofacial muscles in healthy volunteers and patients with primary muscle disorders, they have not yet been systematically studied in patients with a range of neurological conditions.

When applied to muscles of the face and tongue, such tools could 1. Improve accuracy of early diagnosis; 2. Allow monitoring of speech and swallowing dysfunction over time; 3. Help individualize care; and 4. Serve as biomarkers in clinical trials. We propose that QUS and EIM will provide convenient, reliable, clinically meaningful surrogate markers of orofacial dysfunction in a variety of neurologic conditions.

• Study Type: Observational
• Enrollment (Actual): 100

Contacts and Locations

Study Locations

• United States
  • Massachusetts
    • Boston, Massachusetts, United States, 02215
      • Beth Israel Deaconess Medical Center

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 20 years to 90 years (Adult, Older Adult)
• Accepts Healthy Volunteers: Yes

• Sampling Method: Non-Probability Sample

Study Population

Patients will be recruited principally from the BIDMC outpatient clinics and inpatient ward service. Healthy volunteers will be recruited through online advertising.

Description

Inclusion Criteria:

• Established, or clinically probable, neurologic diagnosis with at least the potential for associated bulbar dysfunction
• Age 20-90 years

Exclusion Criteria:

• History or presence of a medical condition that substantially impacts bulbar function
• Age under 20 or over 90 years

Study Plan

How is the study designed?

Design Details

• Observational Models: Other
• Time Perspectives: Cross-Sectional

Number of groups / cohorts

6

Cohorts and Interventions

Group / Cohort
Patients with ALS; No intervention is used in this study. The health of muscle is monitored over time.
Patients with Myasthenia Gravis; No intervention is used in this study. The health of muscle is monitored over time.
Patients with Muscle Disease; No intervention is used in this study. The health of muscle is monitored over time.
Patients with Stroke; No intervention is used in this study. The health of muscle is monitored over time.
Patients with Parkinson's Disease; No intervention is used in this study. The health of muscle is monitored over time.
Healthy Volunteers; No intervention is used in this study. The health of muscle is monitored over time.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Change in Muscle Health over 18 months by Standard Ultrasound	Selected muscles of the face, limbs, trunk, and tongue will be visualized with painless, non-invasive ultrasound; this measure will be performed in healthy volunteers and participants with neurological conditions	Baseline, Month 3, Month 6, Month 9, Month 12, Month 15, and Month 18

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Change in Score on the Sydney Swallow Questionnaire (over 18 months)	Participants with neurological conditions will answer questions about swallowing	Baseline, Month 3, Month 6, Month 9, Month 12, Month 15, and Month 18
Change in Number of Seconds It takes to Repeat Individual Words Ten Times (over 18 months)	Participants with neurological conditions will be asked to repeat "pepper" and "ticker" ten times as quickly as possible	Baseline, Month 3, Month 6, Month 9, Month 12, Month 15, and Month 18
Change in Number of Seconds It Takes to Swallow of 3 ounces of Water (over 18 months)	Participants with neurological conditions will be asked to perform this task if safe	Baseline, Month 3, Month 6, Month 9, Month 12, Month 15, and Month 18
Change in Score on the Amyotrophic Lateral Sclerosis Rating Scale - Revised (ALSFRS-R) (over 18 months)	Participants who have been diagnosed with ALS will answer questions about function, including speech and swallowing	Baseline, Month 3, Month 6, Month 9, Month 12, Month 15, and Month 18
Change in Score on the Quantitative Myasthenia Gravis Scale (over 18 months)	Participants who have been diagnosed with Myasthenia Gravis will be asked to perform certain tasks, such as raising the arm, and the findings will be scored by a research team member	Baseline, Month 3, Month 6, Month 9, Month 12, Month 15, and Month 18
Change in Score on the United Parkinson's Disease Rating Scale (UPDRS) (over 18 months)	Participants who have been diagnosed with Parkinson's Disease will be asked questions about function, including speech and swallowing	Baseline, Month 3, Month 6, Month 9, Month 12, Month 15, and Month 18
Change in Muscle Health by Standard Concentric Needle Electromyography (EMG) (over 18 months)	Participants who have been diagnosed with ALS will be asked if they are willing to participate in standard needle EMG of selected muscles of the face and tongue	Baseline, Month 3, Month 6, Month 9, Month 12, Month 15, and Month 18
Change in Muscle Health by Specialized Electrical Impedance Arrays	Selected muscles of the face, limbs, trunk, and tongue will be measured using painless, specialized electrode arrays; this measure will be performed in healthy volunteers and participants with neurological conditions	Baseline, Month 3, Month 6, Month 9, Month 12, Month 15, and Month 18

Collaborators and Investigators

• Sponsor: Beth Israel Deaconess Medical Center
• Investigators: Principal Investigator: Seward Rutkove, MD, Beth Israel Deaconess Medical Center

Study record dates

Study Major Dates

• Study Start: October 1, 2013
• Primary Completion (Actual): December 31, 2021
• Study Completion (Actual): December 31, 2021

Study Registration Dates

• First Submitted: April 8, 2014
• First Submitted That Met QC Criteria: April 17, 2014
• First Posted (Estimated): April 21, 2014

Study Record Updates

• Last Update Posted (Actual): April 16, 2024
• Last Update Submitted That Met QC Criteria: April 13, 2024
• Last Verified: April 1, 2023

More Information

Terms related to this study

• Keywords: Ultrasound; Stroke; ALS; Parkinson's Disease; Myasthenia Gravis; Myopathy; Bulbar; Orofacial; Electrical Impedance Myography
• Additional Relevant MeSH Terms: Metabolic Diseases; Brain Diseases; Central Nervous System Diseases; Immune System Diseases; Neoplasms; Autoimmune Diseases of the Nervous System; Autoimmune Diseases; Neoplasms by Site; Musculoskeletal Diseases; Neuromuscular Diseases; Parkinsonian Disorders; Basal Ganglia Diseases; Movement Disorders; Synucleinopathies; Neurodegenerative Diseases; Spinal Cord Diseases; TDP-43 Proteinopathies; Proteostasis Deficiencies; Nervous System Neoplasms; Paraneoplastic Syndromes, Nervous System; Paraneoplastic Syndromes; Neuromuscular Junction Diseases; Parkinson Disease; Motor Neuron Disease; Amyotrophic Lateral Sclerosis; Nervous System Diseases; Myasthenia Gravis; Muscular Diseases
• Other Study ID Numbers: 2013P000204