Innovative Measures of Speech and Swallowing Dysfunction in Neurological Disorders

Study Overview
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Brief Summary
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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.

Detailed Description
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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.

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

Conditions
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Amyotrophic Lateral Sclerosis (ALS), Myasthenia Gravis, Myopathies, Stroke, Parkinson's Disease

Participation Criteria
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Eligibility Criteria
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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

Ages Eligible for Study
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Minimum Age: 20 Years
Maximum Age: 90 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Patients with ALS<br>No intervention is used in this study. The health of muscle is monitored over time. | |
| Patients with Myasthenia Gravis<br>No intervention is used in this study. The health of muscle is monitored over time. | |
| Patients with Muscle Disease<br>No intervention is used in this study. The health of muscle is monitored over time. | |
| Patients with Stroke<br>No intervention is used in this study. The health of muscle is monitored over time. | |
| Patients with Parkinson's Disease<br>No intervention is used in this study. The health of muscle is monitored over time. | |
| Healthy Volunteers<br>No intervention is used in this study. The health of muscle is monitored over time. | |

What is the study measuring?
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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 | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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ALS, Myasthenia Gravis, Myopathy, Stroke, Parkinson's Disease, Bulbar, Orofacial, Ultrasound, Electrical Impedance Myography