Pulmonary Function Screening

 

 Buy Article Permissions and Reprints

ABSTRACT

Patients who suffer from neuromuscular diseases often have complications from respiratory insufficiencies. Some neuromuscular diseases, for example Landry Guillain-Barré syndrome, may only require temporary tracheal intubation; patients with other neuromuscular diseases, such as amyotrophic lateral sclerosis, may decide with the assistance of their doctor and family to opt for lifelong noninvasive ventilatory support. Other patients may only opt for noninvasive positive pressure ventilation. Respiratory dysfunction is caused by weakness of the upper airway muscles, which can lead to sleep apnea, abnormal swallow, and decreased respiratory muscle strength, as well as a decrease in total lung volume. Early respiratory changes in patients with neuromuscular disease are often best detected during sleep. During rapid eye movement sleep, there is a reduction in respiratory drive predisposing to hypopneas and apneas. The majority of neuromuscular patients with respiratory insufficiency may be monitored and treated in the outpatient setting, thus allowing them to remain in their homes.

REFERENCES

• 1 Green M, Moxham J. The respiratory muscles.  Clin Sci (Lond) . 1985;  68 1-10
  PubMedGoogle Scholar
• 2 Berger A J. Control of breathing. In: Murray JF, Nadel JA, eds. Textbook of Respiratory Medicine Philadelphia: WB Saunders 1994: 199-210
  Google Scholar
• 3 Krivickas L S. Pulmonary function and respiratory failure. In: Mitsumoto H, Chad DA, Pioro EP, eds. Amyotrophic Lateral Sclerosis Philadelphia: FA Davis Co. 1998: 22:382-404
  Google Scholar
• 4 Griggs R C, Mendell J R, Miller R G. Prevention and management of medical complications of myopathies. In: Evaluation and Treatment of Myopathies Philadelphia: FA Davis Co. 1995 (12): 408-425
  Google Scholar
• 5 Griggs R C, Donohoe K M, Utell M J, Goldblatt D, Moxley III T R. Evaluation of pulmonary function in neuromuscular disease.  Arch Neurol . 1981;  38 9-12
  CrossrefPubMedGoogle Scholar
• 6 Miller R G, Rosenberg J A, Gelinas D F. et al . Practice parameter: the care of the patient with amyotrophic lateral sclerosis (an evidence-based review). Report of the Quality Standards Subcommittee of the American Academy of Neurology.  Neurology . 1999;  52 1311-1323
  PubMedGoogle Scholar
• 7 Oppenheimer E A. Respiratory management and home mechanical ventilation in amyotrophic lateral sclerosis. In: Mitsumoto H, Norris RH Jr, eds. Amyotrophic Lateral Sclerosis: A Comprehensive Guide to Management New York: Demos Medical Publishing 1994: 139-162
  Google Scholar
• 8 Ellis E R, Bye P TP, Bruderer J W. et al . Treatment of respiratory failure during sleep in patients with neuromuscular disease.  Am Rev Resp Dis . 1987;  135 148-152
  PubMedGoogle Scholar
• 9 Bach J R, Alba A S. Management of chronic alveolar hypoventilation by nasal ventilation.  Chest . 1990;  97 52-57
  PubMedGoogle Scholar
• 10 Pinto A, Evangelista T, Calvalho M. et al . Respiratory assistance with a noninvasive ventilator (BiPAP) in motor neuron disease/amyotrophic lateral sclerosis patients: survival rates in a controlled trial.  J Neurol Sci . 1995;  129 19-26
  CrossrefPubMedGoogle Scholar
• 11 Cazzolli P A, Oppenheimer E A. Home mechanical ventilation for amyotrophic lateral sclerosis: nasal compared to tracheostomy-intermittent positive pressure ventilation.  J Neurol Sci . 1996;  139 123-128
  CrossrefPubMedGoogle Scholar
• 12 Borasio G D, Voltz R. Palliative care in amyotrophic lateral sclerosis.  J Neurol . 1997;  244 (suppl 4) S11-S17
  PubMedGoogle Scholar
• 13 Bach J R. Respiratory muscle aids for the prevention of pulmonary morbidity and mortality.  Semin Neurol . 1995;  15 72-83
  PubMedGoogle Scholar
• 14 Tobin M J. Respiratory muscles in disease.  Clin Chest Med . 1988;  9 263-286
  PubMedGoogle Scholar
• 15 Goldstein R S. Hypoventilation: neuromuscular and chest wall disorders.  Clin Chest Med . 1992;  13 507-521
  PubMedGoogle Scholar