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

NCT06841341 | Enrolling By Invitation

• 1 other identifier: JagiellonianU75
• Study Type: observational
• Target: 80
• Locations: 1 country
• Sites: 1

Brief Summary

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.

Conditions

Amyotrophic Lateral Sclerosis

Keywords

ALS; electromyography; ultrasound; respiratory muscles; rectus abdominis; sternocleidomastoideus; diaphragm; vagus; accessorius; noninvasive ventilation; respiratory insufficiency

Outcome Measures

Primary Outcomes (4)

• Relation of denervation in respiratory muscles to PaO2

  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

  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

  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

  Correlation between FSS score and SVC.

  Through study completion, an average of 1 year

Secondary Outcomes (5)

• Correlation of CSA of phrenic nerves with SVC

  Through study completion, an average of 1 year

• Correlation of CSA of accessory nerves with SVC

  Through study completion, an average of 1 year

• Correlation of diaphragm diameter with SVC

  Through study completion, an average of 1 year

• Correlation of daytime sleepiness with SVC

  Through study completion, an average of 1 year

• Correlation of depression with SVC

  Through study completion, an average of 1 year

Study Arms (1)

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; Diagnostic Test: ultrasonography; Diagnostic Test: Arterial blood gas analysis; Diagnostic Test: slow vital capacity

Interventions

electromyography DIAGNOSTIC_TEST

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.

Patients with ALS

ultrasonography DIAGNOSTIC_TEST

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.

Patients with ALS

Arterial blood gas analysis DIAGNOSTIC_TEST

Assessment of partial oxygen (PaO2) and partial carbondioxide pressure (PaCO2) in arterial blood.

Patients with ALS

slow vital capacity DIAGNOSTIC_TEST

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.

Patients with ALS

Eligibility Criteria

• Age: 18 Years - 80 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• 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.

You may qualify if:

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

You may not qualify if:

• 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

Sponsors & Collaborators

• Jagiellonian University: lead

Study Sites (1)

Jagiellonian University Medical College, Department of Neurology

Krakow, Lesser Poland Voivodeship, 31503, Poland

Location

Related Publications (1)

• Brooks BR, Miller RG, Swash M, Munsat TL; World Federation of Neurology Research Group on Motor Neuron Diseases. El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord. 2000 Dec;1(5):293-9. doi: 10.1080/146608200300079536. No abstract available.

  PMID: 11464847 BACKGROUND

MeSH Terms

Conditions

Amyotrophic Lateral Sclerosis; Respiratory Insufficiency

Interventions

Electromyography; Ultrasonography; Blood Gas Analysis

Condition Hierarchy (Ancestors)

Spinal Cord Diseases; Central Nervous System Diseases; Nervous System Diseases; Motor Neuron Disease; Neurodegenerative Diseases; TDP-43 Proteinopathies; Neuromuscular Diseases; Proteostasis Deficiencies; Metabolic Diseases; Nutritional and Metabolic Diseases; Respiration Disorders; Respiratory Tract Diseases

Intervention Hierarchy (Ancestors)

Electrodiagnosis; Diagnostic Techniques and Procedures; Diagnosis; Myography; Diagnostic Imaging; Blood Chemical Analysis; Clinical Chemistry Tests; Clinical Laboratory Techniques; Respiratory Function Tests; Diagnostic Techniques, Respiratory System; Investigative Techniques

Study Design

• Study Type: observational
• Observational Model: CASE CONTROL
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Principal Investigator

Study Record Dates

• First Submitted: February 18, 2025
• First Posted: February 24, 2025
• Study Start: September 8, 2025
• Primary Completion (Estimated): December 31, 2028
• Study Completion (Estimated): June 30, 2029
• Last Updated: September 15, 2025

Record last verified: 2025-09

Data Sharing

• IPD Sharing: Will share
• Shared Documents: STUDY PROTOCOL, ICF
• Time Frame: After the study is completed for the period of ten years.
• Access Criteria: Researchers affiliated in institutions listed on clinicaltrials.gov.

Locations

PL (1)