During this pilot study, the investigators will examine the effects of whole-body electrical muscle stimulation exercise (WB-EMS Exercise) on neuromuscular junction (NMJ) transmission and fatigability in adults with Generalized Myasthenia Gravis (gMG). The investigators will also test whether a relationship exists between NMJ transmission dysfunction and fatigability in gMG, which has long been presumed but never directly assessed. Participants will undergo clinical and electrophysiologic testing before and after the WB-EMS Exercise intervention. The WB-EMS Exercise intervention will be delivered 2 times per week for 4 weeks. Long-term follow up is optional. The hypotheses are (a) that the WB-EMS exercise will improve fatigability and NMJ transmission, and (b) that NMJ transmission dysfunction is related to fatigability.
During this pilot study, the investigators will examine the effects of a novel paradigm, whole-body electrical muscle stimulation exercise (WB-EMS Exercise), on neuromuscular junction (NMJ) transmission and fatigability in adults with Generalized Myasthenia Gravis (gMG). The investigators will also test whether a relationship exists between NMJ transmission dysfunction and fatigability in gMG, which has long been presumed but never directly assessed. The investigators will use single fiber electromyography (SFEMG) to capture pre- and post-treatment jitter, which measures NMJ transmission variability. The investigators will use decomposition electromyography and clinical measures to assess pre- and posttreatment fatigability of motor units and muscles. and associate these with baseline values for NMJ transmission to determine the relationship between these variables. At the conclusion of the study, expected outcomes include: (1) preliminary data regarding the efficacy of WB-EMS Exercise to address fatigability, (2) an indication of whether this type of exercise may promote NMJ remodeling, and (3) clarification of mechanistic connections between NMJ transmission and fatigability. These findings will provide new insights into mechanisms of fatigability and responses to exercise in gMG. The long-term goal is to define the effects of exercise on gMG pathophysiology and identify effective and tolerable modes of exercise that can be recommended to manage and prevent gMG-related fatigue.
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
TREATMENT
Masking
NONE
Enrollment
16
This is a fitness device that delivers whole-body electrical muscle stimulation in conjunction with exercise programs using an iPad App. The stimulation suit (fitted shorts/shirt, vest, leg straps, arm straps) will be donned with the assistance of trained research personnel. Muscle groups stimulated include biceps, triceps, pectorals, abdominals, periscapular musculature, paraspinal musculature, gluteus musculature, quadriceps, and hamstrings. The stimulation level of each individual muscle group can be tailored to participant responses and tolerance (i.e. stimulation of the quadriceps and biceps can occur at different settings). While the participant is following along with the video, a qualified health care professional will be monitoring and adjusting the stimulation levels based on participant responses; rate of perceived exertion will also be monitored to ensure safe moderate intensity levels are maintained.
University of Kansas Clinical Research Center
Fairway, Kansas, United States
RECRUITINGNextGen Precision Health Building, Clinical and Translational Science Unit
Columbia, Missouri, United States
RECRUITINGMean change from baseline in single fiber electromyography (SFEMG) jitter
Single fiber electromyography (SFEMG) jitter is a measure of neuromuscular junction transmission variability; a small needle will be inserted into one of the quadriceps muscles to obtain the measurement
Time frame: Measured within 3 days of starting the intervention and within 3 days of completing the intervention
Mean change from baseline in the linear change in performance during the six-minute walk test (6MWT)
Six-minute Walk Test (6MWT) is a measure of endurance and muscle fatigability; participants will walk as far as they can in 6 minutes
Time frame: Measured within 3 days of starting the intervention and within 3 days of completing the intervention
Mean change from baseline in the linear change in performance during the arm movement test (AMT)
Arm Movement Test (AMT) is a measure of upper extremity muscle fatigability; participants will move their arm back and forth between 2 targets as many times as they can in 90 seconds
Time frame: Measured within 3 days of starting the intervention and within 3 days of completing the intervention
Mean change from baseline in the quantitative myasthenia gravis (QMG) score
Quantitative Myasthenia Gravis (QMG) is a measure of overall disease severity; items include eye movements, swallowing, speaking, breathing test, grip strength, and neck/arm/leg endurance. Thirteen items will be rated on a scale of 0-3 where higher scores indicated greater disease severity.
Time frame: Measured within 3 days of starting the intervention and within 3 days of completing the intervention
Mean change from baseline in motor unit firing rates of the vastus lateralis using decomposition electromyography (dEMG)
Decomposition electromyography (dEMG) is a measurement of motor unit activity; a surface electrode will be placed over the vastus lateralis in the thigh and participants will be asked to activate that muscle
Time frame: Measured within 3 days of starting the intervention and within 3 days of completing the intervention
Mean change from baseline in motor unit firing rates of the middle deltoid using decomposition electromyography (dEMG)
Decomposition electromyography (dEMG) is a measurement of motor unit activity; a surface electrode will be placed over the middle deltoid in the shoulder and participants will be asked to activate that muscle
Time frame: Measured within 3 days of starting the intervention and within 3 days of completing the intervention
Mean change from baseline in fatigue severity scale (FSS)
Fatigue Severity Scale (FSS) is a patient-reported outcome measure assessing the impact of perceived fatigue on a person's life over the prior 7-day period. Nine items are rated on a scale of 1-7 where higher scores indicate more impact of perceived fatigue.
Time frame: Measured within 3 days of starting the intervention and within 3 days of completing the intervention
W. David Arnold, MD
CONTACT
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