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

Condition(s) studied

Investigational drug(s) / intervention(s)

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.

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.

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

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.

Study 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.

Eligibility

Primary outcome measure(s)

• Relation of denervation in respiratory muscles to PaO2 — 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.
• Relation of denervation in respiratory muscles to PaCO2 — 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.
• Relation of denervation in respiratory muscles to SVC — 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.
• Correlation between fatigue and SVC — Through study completion, an average of 1 year
  Correlation between FSS score and SVC.

Trial sites (1)

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