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

Официальное название

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

Обзор

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.

Подробное описание

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.

Вмешательства

• Диагностический тест 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 ef
• Диагностический тест 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.
• Диагностический тест Arterial blood gas analysis
  Assessment of partial oxygen (PaO2) and partial carbondioxide pressure (PaCO2) in arterial blood.
• Диагностический тест 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.

Первичные конечные точки

• Relation of denervation in respiratory muscles to PaO2 [Срок оценки: Through study completion, an average of 1 year]
• Relation of denervation in respiratory muscles to PaCO2 [Срок оценки: Through study completion, an average of 1 year]
• Relation of denervation in respiratory muscles to SVC [Срок оценки: Through study completion, an average of 1 year]
• Correlation between fatigue and SVC [Срок оценки: Through study completion, an average of 1 year]

Вторичные конечные точки (5)

• Correlation of CSA of phrenic nerves with SVC [Срок оценки: Through study completion, an average of 1 year]
• Correlation of CSA of accessory nerves with SVC [Срок оценки: Through study completion, an average of 1 year]
• Correlation of diaphragm diameter with SVC [Срок оценки: Through study completion, an average of 1 year]
• Correlation of daytime sleepiness with SVC [Срок оценки: Through study completion, an average of 1 year]
• Correlation of depression with SVC [Срок оценки: Through study completion, an average of 1 year]

Критерии участия

Критерии включения

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

Критерии исключения

• 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

Критерии приведены из реестра в оригинале (на английском). Окончательную оценку соответствия проводит исследовательский центр.

Здоровые добровольцы: Нет

Дизайн исследования

Модель наблюдения

Случай-контроль

Центры проведения

Польша · 1 центр

• Jagiellonian University Medical College, Department of Neurology — Krakow

Публикации

• 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

Идентификаторы

NCT: NCT06841341 · JagiellonianU75

Первоисточники (государственные реестры)

• ClinicalTrials.gov (NIH, США)

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