Electrophysiology and Ultrasound of Respiratory Muscles and Respective Nerves to Predict Respiratory Insufficiency in ALS

September 8, 2025 updated by: Jakub Antczak, Jagiellonian University

EMG and Ultrasound of Respiratory Muscles and Respective Nerves and to Predict Respiratory Insufficiency in ALS

Respiratory insufficiency is a common complication in amyotrophic lateral sclerosis (ALS). Survival can be prolonged with noninvasive ventilation (NIV). The goal of this study is to assess the value of electromyography of respiratory muscles as well as the ultrasound of the diaphragm and nerves involved in respiration in predicting the onset of respiratory insufficiency, which may improve the timing of NIV start. The study will recruit 80 patients with definite or probable ALS. Investigations include electromyography of bilateral sternocleidomastoideus and rectus abdominis with assessment of presence of acute and chronic denervation. Ultrasonographic measurements include crosssectional area and diameter of bilateral phrenicus, vagus and accesory nerves as well as diameter of diaphragm. Respiratory insufficiency is asessed with slow vital capacity and partial pressure of arterial oxygen and arterial carbon dioxide. Forthermore, ALS severity is assessed with revised ALS Functional Rating Scale and the symptoms which may reflect respiratory insufficiency even in the absence of dyspnea such as sleepiness, fatigue and depression are assessed with Fatigue Severity Scale, Epworth Sleepiness Scale and Hamilton Depression Rating Scale respectively. SVC and blood gases will be compared between group with and without denervation in investigated muscles. SVC and blood gases will be correlated with corssectional areas and diameters of investigated nerves and diaphragm as well as with the scores obtained in used scales and questionnaires.

Study Overview

Status

Status

Indicates the current recruitment status or the expanded access status.
Enrolling by invitation

Conditions

Conditions

The disease, disorder, syndrome, illness, or injury that is being studied. On ClinicalTrials.gov, conditions may also include other health-related issues, such as lifespan, quality of life, and health risks.

Intervention / Treatment

Intervention / Treatment

A process or action that is the focus of a clinical study. Interventions include drugs, medical devices, procedures, vaccines, and other products that are either investigational or already available. Interventions can also include noninvasive approaches, such as education or modifying diet and exercise.

Detailed Description

Amyotrophic lateral sclerosis (ALS) is a rare condition with progressing degeneration of upper and lower motor neurons. Main symptoms include muscle weakness, spasticity, atrophy and fasciculations, which lead to gait impairment, dysphagia, dysarthria and loss of hand dexterity. Currently no disease modifying treatment is available and the condition leads to death, usually within three to five years. Respiratory insufficiency is a common complication, especially in later stages of the disease. It results from weakness of respiratory muscles and in some cases also from chronic aspiration pneumonia. It decreases significantly quality of life and may shorten the survival. Not infrequently it is not accompanied by feeling of dyspnea but may be associated with other symptoms like tiredness, sleepiness, and depression. Survival and the quality of life can be improved with noninvasive ventilation (NIV), but the timing of detection of respiratory insufficiency and starting the therapy is crucial to achieve the best possible efficacy. The goal of this study is to assess the value of electromyography of respiratory muscles as well as the ultrasound of the diaphragm and nerves involved in respiration in predicting the onset of respiratory insufficiency. Electromyography is a method commonly used to confirm the diagnosis and neuromuscular ultrasound it is increasingly used along with electromyography to exclude mimics, especially in atypical cases. Investigators hypothesize, including nerves and muscles involved in respiration into electrophysiological and ultrasonographic testing may yield additional benefit of detecting actual or antecedent respiratory failure. The study will recruit 80 patients with definite or probable ALS. Investigations include electromyography of bilateral sternocleidomastoideus and rectus abdominis with assessment of presence of acute and chronic denervation. Ultrasonographic measurements include cross-sectional area and diameter of bilateral phrenicus, vagus and accessory nerves as well as diameter of diaphragm. Respiratory insufficiency is assessed with slow vital capacity and partial pressure of arterial oxygen and arterial carbon dioxide. Furthermore, ALS severity is assessed with revised ALS Functional Rating Scale and the symptoms which may reflect respiratory insufficiency even in the absence of dyspnea such as sleepiness, fatigue and depression are assessed with Fatigue Severity Scale, Epworth Sleepiness Scale and Hamilton Depression Rating Scale respectively. SVC and blood gases will be compared between group with and without denervation in investigated muscles. SVC and blood gases will be correlated with corssectional areas and diameters of investigated nerves and diaphragm as well as with the scores obtained in used scales and questionnaires.

Study Type

Study Type

Describes the nature of a clinical study. Study types include interventional studies (also called clinical trials), observational studies (including patient registries), and expanded access.
Observational

Enrollment (Estimated)

Enrollment

The number of participants in a clinical study. The "anticipated" enrollment is the target number of participants that the researchers need for the study.
80

Contacts and Locations

This section provides the contact details for those conducting the study, and information on where this study is being conducted.

Study Contact

Study Contact

The name and contact information for the person who can answer enrollment questions for a clinical study. Each location where the study is being conducted may also have a specific contact, who may be better able to answer those questions.

Study Contact Backup

Study Locations

  • Poland
    • Lesser Poland Voivodeship
      • Krakow, Lesser Poland Voivodeship, Poland, 31503
        • Jagiellonian University Medical College, Department of Neurology

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Eligibility Criteria

Eligibility Criteria

The key requirements that people who want to participate in a clinical study must meet or the characteristics they must have. Eligibility criteria consist of both inclusion criteria (which are required for a person to participate in the study) and exclusion criteria (which prevent a person from participating). Types of eligibility criteria include whether a study accepts healthy volunteers, has age or age group requirements, or is limited by sex.

Ages Eligible for Study

  • Adult
  • Older Adult

Accepts Healthy Volunteers

No

Sampling Method

Non-Probability Sample

Study Population

Participants will be recruited from the neurologic ambulatory and neurologic clinic of Jagiellonian University Medical College in Cracow.

Description

Inclusion Criteria:

  • Diagnosis of definite ALS or probable ALS or clinically probable laboratory supported ALS according to revised El Escorial Criteria [Brooks et al. 2000]

Exclusion Criteria:

  • Diagnosis of fronto-temporal dementia
  • Significant cognitive or behavioral deficits of other origin, which may disturb the participation in the study
  • Medical conditions other than SLA likely to cause respiratory insufficiency, significant changes in the blood gases or SVC
  • INR (internal normalized ratio) more than 3 (in the last test performed) in patients with history of coagulation disturbances or intake of oral anticoagulants

Study Plan

This section provides details of the study plan, including how the study is designed and what the study is measuring.

How is the study designed?

Design Details

Cohorts and Interventions

Group / Cohort

Group / Cohort

A group or subgroup of participants in an observational study that is assessed for biomedical or health outcomes.
Intervention / Treatment

Intervention / Treatment

A process or action that is the focus of a clinical study. Interventions include drugs, medical devices, procedures, vaccines, and other products that are either investigational or already available. Interventions can also include noninvasive approaches, such as education or modifying diet and exercise.
Patients with ALS
Patients with definite, probable or laboratory-supported probable ALS according to revised el Escorial criteria, who are treated in the neurologic ambulatory of the University Hospital in Cracow (Poland)
Diagnostic test: electromyography
electromyography of a set of muscles usually examined during diagnosis of ALS (typically tongue, bilateral deltoid, first dorsal interosseur, abductor pollicis brevis, paraspinals, vastus lateralis, anterior tibialis and additionally bilateral sternocleidomastoideus and rectus abdominis). In each muscle recording in relaxation and in maximal voluntary effort will be done. The presence of signs of acute (fibrillations, sharp waves) and chronic (fasciculations, reduction of the recording of the effort) denervation as well as presence of activity coupled with the rhythm of respiration will be assessed.
Diagnostic test: ultrasonography
Ultrasonographic measurements include cross-sectional area (CSA) and diameter of bilateral phrenicus, vagus and accessory nerves as well as diameter of diaphragm. The diameter of diaphragm will be measured during normal, quiet respiration as well as at the peak inspiration and expiration.
Diagnostic test: Arterial blood gas analysis
Assessment of partial oxygen (PaO2) and partial carbondioxide pressure (PaCO2) in arterial blood.
Diagnostic test: slow vital capacity
Slow vital capacity (SVC) will be measured as the full exhalation done after inspiration which will follow a period of quiet breathing. The test will be performed thrice and the final value will be the average value.

What is the study measuring?

Primary Outcome Measures

Primary Outcome Measures

In a clinical study's protocol, the planned outcome measure that is the most important for evaluating the effect of an intervention/treatment. Most clinical studies have one primary outcome measure, but some have more than one.
Outcome Measure
Measure Description
Time Frame
Relation of denervation in respiratory muscles to PaO2
Time Frame: Through study completion, an average of 1 year
Difference in PaO2 between patients with signs of denervation in at least one of the examined respiratory muscles and without signs of denervation.
Through study completion, an average of 1 year
Relation of denervation in respiratory muscles to PaCO2
Time Frame: Through study completion, an average of 1 year
Difference in PaCO2 between patients with signs of denervation in at least one of the examined respiratory muscles and without signs of denervation.
Through study completion, an average of 1 year
Relation of denervation in respiratory muscles to SVC
Time Frame: Through study completion, an average of 1 year
Difference in SVC between patients with signs of denervation in at least one of the examined respiratory muscles and without signs of denervation.
Through study completion, an average of 1 year
Correlation between fatigue and SVC
Time Frame: Through study completion, an average of 1 year
Correlation between FSS score and SVC.
Through study completion, an average of 1 year

Secondary Outcome Measures

Secondary Outcome Measures

In a clinical study's protocol, a planned outcome measure that is not as important as the primary outcome measure for evaluating the effect of an intervention but is still of interest. Most clinical studies have more than one secondary outcome measure.
Outcome Measure
Measure Description
Time Frame
Correlation of CSA of phrenic nerves with SVC
Time Frame: Through study completion, an average of 1 year
Correlation of CSA of phrenic nerve (averaged for both sides) with SVC.
Through study completion, an average of 1 year
Correlation of CSA of accessory nerves with SVC
Time Frame: Through study completion, an average of 1 year
Correlation of CSA of accessory nerve (averaged for both sides) with SVC.
Through study completion, an average of 1 year
Correlation of diaphragm diameter with SVC
Time Frame: Through study completion, an average of 1 year
Correlation of diaphragm diameter (averaged for both sides) with SVC.
Through study completion, an average of 1 year
Correlation of daytime sleepiness with SVC
Time Frame: Through study completion, an average of 1 year
Correlation of ESS score with SVC.
Through study completion, an average of 1 year
Correlation of depression with SVC
Time Frame: Through study completion, an average of 1 year
Correlation of HDRS score with SVC.
Through study completion, an average of 1 year

Collaborators and Investigators

This is where you will find people and organizations involved with this study.

Sponsor

Sponsor

The organization or person who initiates the study and who has authority and control over the study.
Jagiellonian University

Publications and helpful links

The person responsible for entering information about the study voluntarily provides these publications. These may be about anything related to the study.

General Publications

Study record dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Major Dates

Study Start (Actual)

Study Start

The actual date on which the first participant was enrolled in a clinical study. The "anticipated" study start date is the date that the researchers think will be the study start date.
September 8, 2025

Primary Completion (Estimated)

Primary Completion

The date on which the last participant in a clinical study was examined or received an intervention to collect final data for the primary outcome measure. Whether the clinical study ended according to the protocol or was terminated does not affect this date. For clinical studies with more than one primary outcome measure with different completion dates, this term refers to the date on which data collection is completed for all the primary outcome measures. The "anticipated" primary completion date is the date that the researchers think will be the primary completion date for the study.
December 31, 2028

Study Completion (Estimated)

Study Completion

The date on which the last participant in a clinical study was examined or received an intervention/treatment to collect final data for the primary outcome measures, secondary outcome measures, and adverse events (that is, the last participant's last visit). The "anticipated" study completion date is the date that the researchers think will be the study completion date.
June 30, 2029

Study Registration Dates

First Submitted

First Submitted

The date on which the study sponsor or investigator first submitted a study record to ClinicalTrials.gov. There is typically a delay of a few days between the first submitted date and the record's availability on ClinicalTrials.gov (the first posted date).
February 18, 2025

First Submitted That Met QC Criteria

First Submitted That Met QC Criteria

The date on which the study sponsor or investigator first submits a study record that is consistent with National Library of Medicine (NLM) quality control (QC) review criteria. The sponsor or investigator may need to revise and submit a study record one or more times before NLM's QC review criteria are met. It is the responsibility of the sponsor or investigator to ensure that the study record is consistent with the NLM QC review criteria.
February 18, 2025

First Posted (Actual)

First Posted

The date on which the study record was first available on ClinicalTrials.gov after National Library of Medicine (NLM) quality control (QC) review has concluded. There is typically a delay of a few days between the date the study sponsor or investigator submitted the study record and the first posted date.
February 24, 2025

Study Record Updates

Last Update Posted (Estimated)

Last Update Posted

The most recent date on which changes to a study record were made available on ClinicalTrials.gov. There may be a delay between when the changes were submitted to ClinicalTrials.gov by the study's sponsor or investigator (the last update submitted date) and the last update posted date.
September 15, 2025

Last Update Submitted That Met QC Criteria

Last Update Submitted That Met QC Criteria

The most recent date on which the study sponsor or investigator submitted changes to a study record that are consistent with National Library of Medicine (NLM) quality control (QC) review criteria. It is the responsibility of the sponsor or investigator to ensure that the study record is consistent with the NLM QC review criteria.
September 8, 2025

Last Verified

Last Verified

The most recent date on which the study sponsor or investigator confirmed the information about a clinical study on ClinicalTrials.gov as accurate and current. If a study with a recruitment status of recruiting; not yet recruiting; or active, not recruiting has not been confirmed within the past 2 years, the study's recruitment status is shown as unknown.
September 1, 2025

More Information

Terms related to this study

Keywords

Additional Relevant MeSH Terms

Other Study ID Numbers

Other Study ID Numbers

Identifiers or ID numbers other than the NCT number that are assigned to a clinical study by the study's sponsor, funders, or others. These numbers may include unique identifiers from other trial registries and National Institutes of Health grant numbers.
  • JagiellonianU75

Plan for Individual participant data (IPD)

Plan to Share Individual Participant Data (IPD)?

YES

IPD Plan Description

After the study is completed, the scans of collected questionnaires will be available upon request sent to the e-mail: jakub.antczak@uj.edu.pl

IPD Sharing Time Frame

After the study is completed for the period of ten years.

IPD Sharing Access Criteria

Researchers affiliated in institutions listed on clinicaltrials.gov.

IPD Sharing Supporting Information Type

  • STUDY_PROTOCOL
  • ICF

Drug and device information, study documents

Studies a U.S. FDA-regulated drug product

No

Studies a U.S. FDA-regulated device product

No

product manufactured in and exported from the U.S.

No

This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.

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