Respiratory System in Amyotrophic Lateral Sclerosis

Breathing pattern and respiratory mechanics in patients with amyotrophic lateral sclerosis

“…Abnormalities in pulmonary function, including reduced FVC declining progressively over time, are reported [3][4][5]26]. NAKANO et al [27] were among the first to report serial pulmonary function studies in ALS.…”

Associations Between Laryngeal and Cough Dysfunction in Motor Neuron Disease with Bulbar Involvement

“…Contributing factors include respiratory impairments and laryngeal dysfunction. Respiratory dysfunction associated with MND may occur due to impairments of the respiratory musculature, 31 and/or weakness of bulbar muscles that are needed to maintain airway patency. Dystussia is one of several complications of respiratory muscle weakness; others include respiratory failure, dyspnea and orthopnea.…”

“…Dystussia is one of several complications of respiratory muscle weakness; others include respiratory failure, dyspnea and orthopnea. 31,57 Dystussia associated with MND contributes to risk for aspiration and respiratory failure.…”

“…20,30 This is true whether the patient has bulbar and/or limb involvement. Despite having no direct effect on the lungs, respiratory impairments associated with MND can occur in any aspect of the mechanical respiratory system, including respiratory centers located in the medulla and weakness of the respiratory musculature for inspiration and forced expiration 31 , and/or dysfunction of the bulbar muscles that help to maintain upper airway patency. Incoordination of swallowing with breathing may also occur.…”

S-nitrosylated GAPDH mediates neuronal apoptosis induced by amyotrophic lateral sclerosis-associated mutant SOD1G93A

“…ALS has been characterized by progressive and selective loss of upper and lower motor neurons, ultimately leading to death within several years of diagnosis (Braun 1987;Caroscio et al 1987). About 90% of all ALS cases are sporadic (sALS), the cause of which is not clear, while about 10% of all cases are familial (fALS) due to mutations of the multiple genes (Pasinelli & Brown 2006).…”