Breathing disorders in neurodegenerative diseases

Abstract

Neurodegenerative disorders are a diverse group of conditions caused by progressive degeneration of neurons resulting in cognitive, motor, sensory, and autonomic dysfunction, leading to severe disability and death. Pulmonary dysfunction is relatively common in these conditions, may be present early in the disease, and is less well recognized and treated than other symptoms. There are variable disorders of upper and lower airways, central control of ventilation, strength of respiratory muscles, and breathing during sleep which further impact daily activities and quality of life and have the potential to injure vulnerable neurons. Laryngopharyngeal dysfunction affects speech, swallowing, and clearance of secretions, increases the risk of aspiration pneumonia, and can cause stridor and sudden death. In Parkinson's disease, L-Dopa benefits some pulmonary symptoms but there are limited pharmacological treatment options for pulmonary dysfunction. Targeted treatments include strengthening of respiratory muscles, positive airway pressure in sleep and techniques to improve cough efficacy. Well-designed clinical trials are needed to evaluate the long-term benefits of these interventions. Challenges for the future include earlier identification of pulmonary dysfunction in the clinic, institution of the most effective treatments (based on clinical trials that measure long-term meaningful outcomes) and the development of neuroprotective treatment.

Keywords: Airway disease; Brainstem control of respiration; Laryngopharyngeal dysfunction; Neurodegenerative disorders; Obstructive sleep apnea; Positive airway pressure in sleep; Pulmonary dysfunction; Respiratory muscle strength training.