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

Sponsor
Jagiellonian University
Study ID
NCT06841341
Status
Enrolling By Invitation

Conditions

Eligibility Criteria

Sex
ALL
Age
18 Years - 80 Years
Healthy Volunteers
Not accepted

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.
  • 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.
  • Arterial blood gas analysis — DIAGNOSTIC_TEST
    Assessment of partial oxygen (PaO2) and partial carbondioxide pressure (PaCO2) in arterial blood.
  • 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.

Study Details

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.

Key Dates

First listed
Feb 24, 2025
Start date
Sep 8, 2025
Status verified
Sep 2025
Primary completion
Dec 31, 2028
Completion
Jun 30, 2029

Study Design

Enrollment
80 participants (estimated)

Arms

  • Arm: 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)

Primary Outcome Measure

Relation of denervation in respiratory muscles to PaO2 [ Time Frame: Through study completion, an average of 1 year ]

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