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Innovative Biomarkers of Orofacial Dysfunction in Amyotrophic Lateral Sclerosis

肌萎缩侧索硬化症口面部功能障碍的创新生物标志物

基本信息

批准号：
9032986
负责人：
Courtney McIlduff
金额：
$ 6.64万
依托单位：
BETH ISRAEL DEACONESS MEDICAL CENTER
依托单位国家：
美国
项目类别：
财政年份：
2015
资助国家：
美国
起止时间：
2015-03-02 至 2017-03-01
项目状态：
已结题

来源：
https://reporter.nih.gov/project-details/9032986
关键词：
Acoustics Age Amyotrophic Lateral Sclerosis Anterior Horn Cells Architecture Atrophic Biological Markers Breathing Cerebrospinal Fluid Proteins Clinical Trials Cognitive Communities Connective Tissue Control Groups Data Deglutition Development Diagnosis Disease Evaluation Evolution Face Fatty acid glycerol esters Fiber Foundations Frequencies Functional disorder Gender Goals Gray unit of radiation dose Health Health Status Image Individual Inflammation Iowa Lead Limb structure Lip structure Measurement Measures Methods Movement Muscle Myography Myopathy Natural History Neuromuscular Diseases Onset of illness Outcome Painless Patients Performance Peripheral Nervous System Diseases Procedures Questionnaires Reproducibility Serum Speech Speed Symptoms System Technology Testing Therapeutic Agents Therapeutic Clinical Trial Therapeutic Trials Time Tongue Training Translating Ultrasonography Visit Work aged base career design effective intervention electric impedance global health healthy volunteer innovation innovative technologies instrument interest meetings member nervous system disorder novel therapeutics orofacial public health relevance response tool user-friendly voltage

项目摘要

DESCRIPTION (provided by applicant): Bulbar dysfunction inevitably develops in the course of amyotrophic lateral sclerosis (ALS), and approximately 25% of patients have predominantly bulbar symptoms at the time of disease onset. In addition to characterizing the evolution in muscle architecture that could underlie associated orofacial weakness, identifying new biomarkers is critical to the development and testing of novel therapeutic agents. As painless, non-invasive, portable technologies, quantitative ultrasonography (QUS) and electrical impedance myography (EIM) could meet the need for objective measures of bulbar dysfunction. In QUS, acoustic energy is applied to a muscle of interest; the resultant raw gray-scale or backscatter pictorial data are translated into a single value that reflects the health of the image muscle. Similarly, in EIM, a high-frequency, low-intensity alternating electrical current is applie 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 the ALS community. We hypothesize that quantitative ultrasonography (QUS) and electrical impedance myography (EIM) measurements can provide consistent, clinically meaningful information about orofacial muscle health in ALS. In a longitudinal natural history study of 32 individuals with ALS and 32 healthy subjects, we will test this hypothesis via three aims: 1.) To assess the reliability of orofacial QS and EIM in individuals with and without ALS; 2) To determine how the measurements of selected orofacial muscles in individuals with ALS evolve over an 18-month period; 3) To evaluate preliminarily whether orofacial QUS and EIM measurements in ALS patients correlate with results of functional tests. If QUS and EIM do provide reliable, meaningful surrogate information about ALS status as expected, they could be used to make proof-of- concept therapeutic trials more efficient. Furthermore, the innovative technologies could have broader application to the diagnosis and surveillance of other central and peripheral nervous system disorders with bulbar involvement.