Amyotrophic Lateral Sclerosis, Healthy, Muscular Dystrophies, Obstructive Sleep Apnea
Conditions
Brief summary
This is a pilot study to examine the diagnostic utility of a novel transmembrane surface sensor, and compare signals obtained with the transmembrane sensor to conventional needle EMG signals from healthy volunteers to those with documented neurologic pharyngeal muscle dysfunction (ALS and muscular dystrophy) and to those with severe OSA.
Detailed description
Transmembrane electromyography (TM-EMG) may be a feasible and valid non-invasive EMG technique for detecting neuromuscular (NM) impairment. This study will assess whether, in healthy volunteers and participants with known obstructive sleep apnea (OSA) and other NM diseases involving the oropharynx, the same characteristic motor unit potentials obtained on conventional needle EMG (NEMG) can be obtained using a TM-EMG sensor. The purpose of this study is to demonstrate whether the TM-EMG sensor can provide the same diagnostic accuracy as the concentric needle electrode for the diagnosis of NM diseases. Having demonstrated diagnostic similarity of TM-EMG to NEMG, the secondary aim of this study is to confirm that NM disturbance of oropharyngeal striated muscles in participants with OSA can be elicited with the TM-EMG sensor.
Interventions
DEVICETransmembrane EMG Oropharynx Probe
Examination of the electromyographic signal from oropharyngeal muscles obtained using an investigational transmembrane sensor attached to a rigid probe and an FDA-approved very fine concentric needle electrode (Ambu Neuroline 25 mm x 30G).
Sponsors
Powell Mansfield Inc.
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
DIAGNOSTIC
Masking
NONE
Intervention model description
This is a prospective, cohort, pilot study with blinded data analysis and two physician testers to assess intertester reliability and device usability. Volunteers will include healthy adults, participants with documented neurologic pharyngeal muscle dysfunction (ALS and muscular dystrophy), and participants with severe OSA. For each participant diagnostic properties of EMG studies will be assessed using a conventional needle and TM-EMG sensor in pharyngeal muscles.
Eligibility
Sex/Gender
ALL
Age
18 Years to 70 Years
Healthy volunteers
Yes
Inclusion criteria
* Age: 18-70 * Must be able to pause use of anticoagulation, NSAID and multi-vitamins for appropriate period prior to study test. * Must be willing to stop any type of smoking or vaping 10 days prior to testing A cohort of participants with documented neurological disorders involving upper airway striated muscles including ALS and muscular dystrophy with the presence of bulbar symptoms. A cohort of participants diagnosed with moderate to severe OSA proven by an in-lab PSG, including the following criteria: * AHI \> 25 * Nadir SaO2 \< 85% * not currently using CPAP A cohort of healthy participants that meet the following criteria: * Normal craniofacial anatomy * BMI \< 30
Exclusion criteria
* Allergy to topical anesthetic * 4 or more alcoholic drinks on the same occasion on 5 or more days in the past month * Prior cancer, or radiation to the head or neck * Craniofacial anatomical disorders * Presence of any underlying medical, surgical or psychiatric disorder that would preclude participation as determined by the principal investigator
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Proof of Diagnostic Consistency | 1 hour | Proof of diagnostic consistency using both the TM-EMG sensor and NEMG in neuromuscular disorders of the oropharyngeal muscles. Significant Inter rater reliability between two blinded neuromuscular experts when using TM-EMG in the palatoglossal and genioglossus. |
Countries
United States
Participant flow
Participants by arm
| Arm | Count |
|---|---|
| Normal Subjects A group of subjects that have been confirmed to have normal oropharyngeal anatomy by an ENT, and also had an overnight in lab PSG with an AHI \< 2. | 6 |
| Amyotrophic Lateral Sclerosis Patients having a confirmed diagnosis of Amyotrophic Lateral Sclerosis (ALS) with bulbar involvement. | 5 |
| Obstructive Sleep Apnea (OSA) Subjects with a confirmed diagnosis of OSA confirmed by in-laboratory polysomnography (PSG) with an AHI of 25 or higher. | 5 |
| Total | 16 |
Baseline characteristics
| Characteristic | Amyotrophic Lateral Sclerosis | Obstructive Sleep Apnea (OSA) | Normal Subjects | Total |
|---|---|---|---|---|
| Age, Categorical <=18 years | 0 Participants | 0 Participants | 0 Participants | 0 Participants |
| Age, Categorical >=65 years | 2 Participants | 2 Participants | 0 Participants | 4 Participants |
| Age, Categorical Between 18 and 65 years | 3 Participants | 3 Participants | 6 Participants | 12 Participants |
| Age, Continuous | 59.20 years STANDARD_DEVIATION 11.63 | 53.20 years STANDARD_DEVIATION 11.41 | 23.66 years STANDARD_DEVIATION 3.9 | 45.47 years STANDARD_DEVIATION 18.18 |
| Race and Ethnicity Not Collected | — | — | — | 0 Participants |
| Region of Enrollment United States | 5 participants | 5 participants | 6 participants | 16 participants |
| Sex: Female, Male Female | 2 Participants | 1 Participants | 5 Participants | 8 Participants |
| Sex: Female, Male Male | 3 Participants | 4 Participants | 1 Participants | 8 Participants |
Adverse events
| Event type | EG000 affected / at risk | EG001 affected / at risk | EG002 affected / at risk |
|---|---|---|---|
| deaths Total, all-cause mortality | 0 / 6 | 0 / 5 | 0 / 5 |
| other Total, other adverse events | 0 / 6 | 0 / 5 | 0 / 5 |
| serious Total, serious adverse events | 0 / 6 | 0 / 5 | 0 / 5 |
Outcome results
Primary
Proof of Diagnostic Consistency
Proof of diagnostic consistency using both the TM-EMG sensor and NEMG in neuromuscular disorders of the oropharyngeal muscles. Significant Inter rater reliability between two blinded neuromuscular experts when using TM-EMG in the palatoglossal and genioglossus.
Time frame: 1 hour
| Arm | Measure | Value (NUMBER) |
|---|---|---|
| Normal Subjects | Proof of Diagnostic Consistency | 95.7 % agreement |
| Amyotrophic Lateral Sclerosis | Proof of Diagnostic Consistency | 78.1 % agreement |
| Obstructive Sleep Apnea (OSA) | Proof of Diagnostic Consistency | 90 % agreement |