Study of Electrical Impedance Myography (EIM) in ALS

Noninvasive Assessment of Neuromuscular Disease Using Electrical Impedance

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.

Study Overview

• Status: Completed
• Conditions: Multiple Sclerosis; Charcot-Marie-Tooth Disease; Motor Neuron Disease; Amyotrophic Lateral Sclerosis
• Intervention / Treatment: Device: Electrical impedance myography (EIM)

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.

• Study Type: Observational
• Enrollment (Actual): 106

Contacts and Locations

Study Locations

• United States
  • Arizona
    • Phoenix, Arizona, United States, 85013
      • St. Joseph's Hospital & Medical Center
  • Florida
    • Miami, Florida, United States, 33136
      • University of Miami Miller School of Medicine
  • Massachusetts
    • Boston, Massachusetts, United States, 02114
      • Massachusetts General Hospital
    • Boston, Massachusetts, United States, 02210
      • Skulpt, Inc
  • New York
    • Syracuse, New York, United States, 13210
      • SUNY Upstate Medical University
  • North Carolina
    • Winston-Salem, North Carolina, United States, 27157
      • Wake Forest University Health Sciences

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 35 years to 80 years (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

People with ALS People diagnosed with early ALS (possible, probable, probable- laboratory supported or definite ALS according to El Escorial criteria)

Other Neurological Diseases People with a diagnosis of a disease that mimics ALS

Healthy Controls Healthy Volunteers that do not have ALS or another neurological disease that mimics ALS.

Description

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 Exclusion Criteria:

• Diagnosis of possible, probable, probable-laboratory supported, or definite ALS
• Presence of positive family history of ALS.
• 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.

Healthy Volunteer Inclusion Criteria:

- Absence of a known neurological disorder.

Healthy Volunteer Exclusion Criteria:

• History of ALS, myopathy, neuropathy, ALS mimic disorder or other neurodegenerative disease.
• Presence of positive family history of ALS.
• 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.

*Please note that this is not a complete listing on all eligibility criteria.*

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Prospective

Number of groups / cohorts

3

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
People with ALS; People diagnosed with early ALS (possible, probable, probable-laboratory supported or definite ALS according to El Escorial criteria) Intervention: Electrical Impedance Myography (EIM).	Device: Electrical impedance myography (EIM); 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.; Other Names: EIM1102 device
Other Neurological Diseases; People with a diagnosis of a disease that mimics ALS	Device: Electrical impedance myography (EIM); 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.; Other Names: EIM1102 device
Healthy Controls; Healthy Volunteers that do not have ALS or another neurological disease that mimics ALS.	Device: Electrical impedance myography (EIM); 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.; Other Names: EIM1102 device

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Discrimination between Groups	Determine EIM device's ability to discriminate between ALS and "look-alike" non-fatal, motor-predominant syndromes	Duration of the Study (9 months for Group A, one visit for Groups B and C)

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Tracking Progression	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	Duration of Study, (9 months for Group A, one visit for Groups B and C)
Correlation with Outcome Measures	Determine whether EIM progression is predictive of a combined outcome of survival and progression as measured by ALSFRS-R, HHD and VC.	Duration of Study (9 months for Group A, one visit for Groups B and C)

Collaborators and Investigators

• Sponsor: Skulpt, Inc.
• Investigators: Principal Investigator: Jeremy Shefner, MD, PhD, State University of New York - Upstate Medical University

Study record dates

Study Major Dates

• Study Start: November 1, 2013
• Primary Completion (Actual): March 1, 2016
• Study Completion (Actual): March 1, 2016

Study Registration Dates

• First Submitted: December 3, 2013
• First Submitted That Met QC Criteria: December 9, 2013
• First Posted (Estimate): December 13, 2013

Study Record Updates

• Last Update Posted (Estimate): May 11, 2016
• Last Update Submitted That Met QC Criteria: May 10, 2016
• Last Verified: May 1, 2016

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Pathologic Processes; Metabolic Diseases; Central Nervous System Diseases; Nervous System Diseases; Immune System Diseases; Demyelinating Autoimmune Diseases, CNS; Autoimmune Diseases of the Nervous System; Demyelinating Diseases; Autoimmune Diseases; Congenital Abnormalities; Genetic Diseases, Inborn; Neuromuscular Diseases; Stomatognathic Diseases; Neurodegenerative Diseases; Peripheral Nervous System Diseases; Spinal Cord Diseases; TDP-43 Proteinopathies; Proteostasis Deficiencies; Heredodegenerative Disorders, Nervous System; Nervous System Malformations; Polyneuropathies; Multiple Sclerosis; Sclerosis; Motor Neuron Disease; Amyotrophic Lateral Sclerosis; Tooth Diseases; Nerve Compression Syndromes; Charcot-Marie-Tooth Disease; Hereditary Sensory and Motor Neuropathy
• Other Study ID Numbers: 2013P001505