Diagnostics and management techniques in respiratory disorders in amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis (ALS) is a rapidly progressive disease characterized by relentless increase in weakness of limb and respiratory muscles. Whereas the etiology is still not clear, there have been a remarkable progress in understanding ALS pathogenesis and pathophysiology over the last years.  In 2017 the second drug that can alter ALS progression rate has been registered. Nevertheless, the prognosis remains very poor and the average life expectancy in ALS does not exceed 5 years. The most common causes of death in ALS are respiratory complications and respiratory failure. Consequently, monitoring of the respiratory system function in ALS patients by neurologists and pulmonologists is crucial for survival and prolongation of life in this condition. Currently the most available diagnostic tool for the assessment of the respiratory profile in ALS is spirometry. Other methods, such as measurement of sniff nasal pressure, overnight oximetry and polysomnography, are also important but, due to technical complexity, their use in routine practice is limited. The principal method of respiratory support used in ALS is noninvasive lung ventilation (NIV). It can increase the average life expectancy and improve the patients’ quality of life. Invasive lung ventilation with endotracheal tube is another technique that can increase life expectancy and improve quality of life. However, the number of patients accepting this type of mechanical ventilation is very low. Multidisciplinary approach involving different medical specialists including neurologists, pulmonologists and critical care physicians is the key to successive management of ALS patients with respiratory disorders.