Assessment of respiratory functions by spirometry and phrenic nerve studies in patients of amyotrophic lateral sclerosis

Objective: Spirometry is the most common test recommended to monitor respiratory dysfunction in patients of amyotrophic lateral sclerosis (ALS). However, the test depends on the patient's efforts and may be difficult to conduct in patients with faciobulbar weakness. We aimed to study the role of phrenic nerve-electrophysiological studies to predict respiratory dysfunction and correlate it with the forced vital capacity (PVC) in patients of ALS. Methods: Forty-three unselected patients (32 male, 25 with limb-onset ALS, age 50 +/- 15 years) with clinically definite or probable ALS were included. They were evaluated at entry and after a period of 6 months with the ALS functional rating scale (ALSFRS), their respiratory subscores (ALS-FRSr), their FVC values as determined by spirometry, and phrenic nerve studies. Results: Six patients could not perform a satisfactory spirometry at the onset and during the course of illness. All the six patients had severe faciobulbar weakness. Respiratory abnormalities on spirometry were found in 85% of patients, whereas only 30% were symptomatic for respiratory dysfunction. In patients with severe respiratory dysfunction (FVC<60%), the phrenic nerve motor amplitudes (PNAMPs) were significantly reduced compared to those with mild-to-moderate respiratory dysfunction (FVC >= 60%). The FVC value showed a significant correlation with the PN-AMP. Nine patients had a poor outcome (death or severe disability) at the end of a period of 6 months. Low levels of both FVC and PN-AMP were predictors of poor outcome for patients at the end of 6 months. Conclusion: We conclude that respiratory dysfunction, as determined by spirometry, is common in patients of ALS. However, only about one-third of patients show symptoms of respiratory distress. Clinical symptoms of respiratory distress are unreliable predictors of respiratory failure in ALS. Measurement of PN-AMP at the time of presentation may be an additional tool to assess respiratory dysfunction in ALS. Reduced PN-AMP values may be indicative of low PVC and may have some role in the assessment of respiratory function in patients in whom a routine spirometry is not possible due to limitations arising from the illness. Both low FVC and reduced PN-AMP at the time of presentation are predictors of poor outcome for patients at the end of 6 months. (C) 2011 Elsevier B.V. All rights reserved.