Affiliations: Division of Pulmonary and Critical Care Medicine, Lankenau Hospital, 100 Lancaster Avenue, MOBW 230, Wynnewood, PA 19096, USA. Tel.: +1 610 642 3796; Fax: +1 610 642 2943; E-mail: gregorys@mlhs.org | Department of Neurology, University of Pennsylvania School of Medicine, Penn Neurological Institute, 330 South 9th Street, Philadelphia, PA 19107, USA
Abstract: Neuromuscular respiratory failure is the cause of death in the majority of patients with ALS. Respiratory muscle dysfunction impacts on quality of life and survival. Attentive management of respiratory muscle weakness is an important aspect of the management of the ALS patient. The respiratory muscles may be thought of as four functional groups: the inspiratory muscles, the expiratory muscles, the accessory muscles of respiration, and the upper airway muscles. This paper will review the structure and function of the neuromuscular respiratory system, and the evaluation and management of respiratory muscle dysfunction in ALS patients.
