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Primary Lateral Sclerosis

No intervention is used in this study. The health of muscle is monitored over time.

Seward Rutkove, MD

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.

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.

Innovative Measures of Speech and Swallowing Dysfunction in Neurological Disorders

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

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

Participants with neurological conditions will answer questions about swallowing

Participants with neurological conditions will be asked to repeat "pepper" and "ticker" ten times as quickly as possible

Participants with neurological conditions will be asked to perform this task if safe

Participants who have been diagnosed with ALS will answer questions about function, including speech and swallowing

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

Participants who have been diagnosed with Parkinson's Disease will be asked questions about function, including speech and swallowing

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

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

Study title	NCT ID	Conditions	Interventions	Eligibility	Organization	Link
Innovative Measures of Speech and Swallowing Dysfunction in Neurological Disorders	NCT02118805	Amyotrophic Lat... Myasthenia Grav... Myopathies Stroke Parkinson's Dis...		20 Years - 90 Years	Beth Israel Deaconess Medical Center
Innovative Measures of Speech and Swallowing Dysfunction in Neurological Disorders	NCT02118805	Amyotrophic Lat... Myasthenia Grav... Myopathies Stroke Parkinson's Dis...		20 Years - 90 Years	Beth Israel Deaconess Medical Center

Spots Global Cancer Trial Database for myopathies

Every month we try and update this database with for myopathies cancer trials from around the world to help patients and their families find trials that might be right for them.
We offer this 100% free of charge and do not endorse any of the trials listed here, we hope it helps you or a loved one.

Spots Global Cancer Trial Database for myopathies

Every month we try and update this database with for myopathies cancer trials from around the world to help patients and their families find trials that might be right for them. We offer this 100% free of charge and do not endorse any of the trials listed here, we hope it helps you or a loved one.

Every month we try and update this database with for myopathies cancer trials from around the world to help patients and their families find trials that might be right for them.
We offer this 100% free of charge and do not endorse any of the trials listed here, we hope it helps you or a loved one.