Amyotrophic Lateral Sclerosis, Charcot-Marie-Tooth Disease, Motor Neuron Disease, Multiple Sclerosis
Conditions
Brief summary
This trial is studying Electrical Impedance Myography (EIM) for measuring muscle health. The trial is studying people with Amyotrophic Lateral Sclerosis (ALS), other neuromuscular diseases, and healthy volunteers to see if the EIM device can measure disease in muscle tissue.
Detailed description
This is a multicenter, 9-month study evaluating the effectiveness of electrical impedance myography (EIM) as a diagnostic and disease-tracking tool. In addition, the following will be studied: 1. Determine EIM device's ability to discriminate between ALS and look-alike non-fatal, motor-predominant syndromes; 2. Track EIM progression over time and determine the best summary EIM measure that could serve as an endpoint in future clinical trials and individual patient care; and, 3. Determine whether EIM progression is predictive of a combined outcome of survival and progression as measured by ALS Functional Rating Scale, Revised (ALSFRS-R), Hand-held Dynamometry (HHD) and Vital Capacity (VC) measures.
Interventions
In EIM, high-frequency alternating electrical current is applied to localized areas of muscle via surface electrodes and the consequent surface voltage patterns analyzed. EIM is very sensitive to the compositional and structural elements of muscle. Data from both human subjects and animal disease models, including ALS, spinal muscular atrophy (SMA), and Duchenne muscular dystrophy (DMD), show that EIM may be sensitive to a variety of pathological states. It is anticipated that EIM will thus likely be able to assist in quantifying the severity of the disease affecting various muscle groups as well as in measuring changes in the disease over time.
Sponsors
Skulpt, Inc.
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
35 Years to 80 Years
Healthy volunteers
Yes
Inclusion criteria
Early ALS Inclusion Criteria: * Sporadic or familial ALS (as defined by revised El Escorial criteria) * Onset of weakness or spasticity due to ALS ≤ 36 months prior to the Screening/Baseline Visit. * Slow vital capacity (SVC) ≥60% of predicted for gender, height, and age Early ALS
Exclusion criteria
\- The presence of unstable psychiatric disease, cognitive impairment, or dementia that would impair ability of the subject to provide informed consent, or a history of active substance abuse within the prior year. ALS Disease Mimics Inclusion Criteria: \- Diagnosis of one of the following: a. Pure Lower Motor Neuron Disease (LMND) mimics: i. Multi-focal motor neuropathy ii. Autoimmune motor neuropathy iii. Cervical or lumbosacral radiculopathies with weakness involving more than one extremity or more than a single myotome if restricted to one extremity. iv. Multiple peripheral mononeuropathies with clinical weakness v. Charcot-Marie-Tooth Disease vi. Any condition that produces generalized or localized weakness without concomitant sensory symptoms, including myasthenia gravis or myopathy, that the evaluating physician deems mimics ALS. b. Pure Upper Motor Neuron Disease (UMND) mimics: i. Cervical myelopathy ii. Multiple sclerosis iii. Hereditary spastic paraparesis ALS Disease Mimics
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Discrimination between Groups | Duration of the Study (9 months for Group A, one visit for Groups B and C) | Determine EIM device's ability to discriminate between ALS and look-alike non-fatal, motor-predominant syndromes |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Tracking Progression | Duration of Study, (9 months for Group A, one visit for Groups B and C) | Track EIM progression over time and determine the best summary EIM measure that could serve as an endpoint in future clinical trials and individual patient care |
| Correlation with Outcome Measures | Duration of Study (9 months for Group A, one visit for Groups B and C) | Determine whether EIM progression is predictive of a combined outcome of survival and progression as measured by ALSFRS-R, HHD and VC. |
Countries
United States
Outcome results
None listed