Pulmonary function testing in neuromuscular disease

Pulmonary function testing is useful in the diagnosis and management of patients with neuromuscular disease. It is important, however, to keep in mind that certain tests commonly used to assess these patients, such as MIPs and MEPs, although useful, are fraught with potential error and rigorous attention should be paid to technical details when performing them. In addition, many studies have shown that pulmonary impairment does not always parallel generalized muscle impairment and thorough testing therefore should be done in any patient with neuromuscular disease to assess the level of respiratory compromise accurately. In addition, the clinician should be aware that the pattern of involvement-bulbar versus inspiratory, versus expiratory muscle weakness-may vary markedly among patients, even with the same diagnosis, so testing should be tailored to detect these patterns. Furthermore, serial follow-up examinations should be performed to track the rate of deterioration so that therapeutic interventions can be initiated before respiratory crises occur.