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

NCT ID: NCT06841341

Last Updated: 2025-09-15

Basic Information

• Recruitment Status: ENROLLING_BY_INVITATION
• Total Enrollment: 80 participants
• Study Classification: OBSERVATIONAL
• Study Start Date: 2025-09-08
• Study Completion Date: 2029-06-30

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.

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.

Conditions

Amyotrophic Lateral Sclerosis

Study Design

• Observational Model Type: CASE_CONTROL
• Study Time Perspective: PROSPECTIVE

Study Groups

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)

electromyography

Intervention Type: 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.

ultrasonography

Intervention Type: 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.

Arterial blood gas analysis

Intervention Type: DIAGNOSTIC_TEST

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

slow vital capacity

Intervention Type: 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.

Interventions

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.

Intervention Type: 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.

Intervention Type: DIAGNOSTIC_TEST

Arterial blood gas analysis

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

Intervention Type: 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.

Intervention Type: DIAGNOSTIC_TEST

Eligibility Criteria

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

• Minimum Eligible Age: 18 Years
• Maximum Eligible Age: 80 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

Jagiellonian University

OTHER

Sponsor Role: lead

Responsible Party

Jakub Antczak

Principal Investigator

Responsibility Role: PRINCIPAL_INVESTIGATOR

Locations

Jagiellonian University Medical College, Department of Neurology

Krakow, Lesser Poland Voivodeship, Poland

Countries

Poland

References

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.

Reference Type: BACKGROUND

PMID: 11464847 (View on PubMed)

Other Identifiers

JagiellonianU75

Identifier Type: -

Identifier Source: org_study_id