Day and night respiratory problems in ALS cases

Introduction: Amyotrophic Lateral Sclerosis (ALS) is a rapidly progressive fatal neuromuscular disease. Survival is approximately 1-3 years after initial diagnosis. Patients usually die due to respiratory failure secondary to respiratory muscle weakness. Respiratory muscles can be affected at any stage of the disease. The frequency of respiratory problems and predictors of mortality are not clear. The aim of this study was to evaluate respiratory problems that may develop during day and night in ALS patients, to investigate predictors of these problems and to evaluate the factors affecting mortality. Method: The medical records of patients who were admitted to the Pulmonary Department outpatient clinic of Istanbul University, Istanbul Medical Faculty with the diagnosis of ALS were evaluated retrospectively. Informations (symptoms, forced vital capacity-FVC, maximum inspiratory and expiratory pressures, sniff nasal inspiratory pressure, peak cough flow-PCF, arterial blood gases) at the first admission to the clinic, sleep studies, follow-up informations and survival were recorded. Results: On admission, 327 patients (180 M, 147 K, age 60.31 ± 11.56 years) had low respiratory function measurements, 14.68% had paradoxical abdominal respiration, 14.98% had hypercapnia, 35.17% had diaphragm dysfunction. During follow-up, 13.46% had never come to control. 64.71% had obstructive sleep apnea. One-year survival was 81.3%, three-year survival was 52.2% and 5-year survival was 37.1%. Survival was better in patients with age≤50, limb onset, ambulatory, FVC> 50%, PCF> 270 L/min, and without diaphragm dysfunction. Conclusion: Respiratory problems are common in patients with ALS at first admission. Age, initial type of the disease, ambulatory/non-ambulatory status, FVC, PCF and diaphragm dysfunction affect survival.