Pulmonary function tests and diaphragmatic compound muscle action potential in patients with sporadic amyotrophic lateral sclerosis

Acta Neurol Scand. 2010 Jun;121(6):400-5. doi: 10.1111/j.1600-0404.2009.01199.x. Epub 2010 Jan 12.


Background: Respiratory failure is the primary cause of death in patients with amyotrophic lateral sclerosis (ALS). Diaphragmatic compound muscle action potentials (DCMAP) are valid parameters to assess the respiratory muscle innervation.

Aim: In this study we propose to establish evidence of pulmonary dysfunction in patients with ALS and its relation to DCMAP parameters among patients with sporadic ALS.

Materials and methods: Twenty nine patients (M-20, F-9) diagnosed to have sporadic ALS by El. Escorial criteria, without symptoms of pulmonary dysfunction, and able to perform the PFT satisfactorily, were studied. Thirty controls (M-20, F-10) were selected from patient's relatives. Forced vital capacity (FVC), forced expiratory volume in one second (FEV(1)), peak expiratory flow rate (PEFR) and maximum voluntary ventilation (MVV) were measured by spirometry. Maximum expiratory pressure (MEP) was measured by digital peak pressure monitor. Right phrenic nerve conductions (DCMAP) were performed and the latencies and amplitude of diaphragmatic com-pound action potential (DCMAP) was recorded in controls and ALS patients.

Results: The mean age of patients was 51.41 +/- 10.72 years (37-82) and control was 53.57 +/- 8.85 years (30-68). None of the patients had symptoms or clinical evidence of respiratory dysfunction. The FVC, FEV1, PEFR, MVV, MIP and MEP were significantly (P < 0.001) reduced in ALS. The mean DCMAP amplitude was reduced among patients (610 +/- 506.231 muv) as compared to controls (1303.33 +/- 584.56, P < 0.001) and mean latency was increased in patients (9.73 +/- 2.57 ms) compared to controls (7.69 +/- 0.87, P = 0.001). There was significant negative correlation between PFTs and latencies of DCMAP. Amplitude of DCMAP did not correlate with PFTs.

Conclusion: There is significant negative correlation between DCMAP latencies and PFTs suggesting early loss of myelinated fibres and diaphragmatic dysfunction. DCMAP latencies may be a good indicator of early respiratory muscle involvement and also of disease progression in ALS.

MeSH terms

  • Action Potentials / physiology*
  • Adult
  • Aged
  • Aged, 80 and over
  • Amyotrophic Lateral Sclerosis / complications*
  • Amyotrophic Lateral Sclerosis / pathology*
  • Diaphragm / physiopathology*
  • Female
  • Forced Expiratory Volume
  • Humans
  • Male
  • Middle Aged
  • Respiratory Function Tests / methods*
  • Respiratory Insufficiency / diagnosis
  • Respiratory Insufficiency / etiology*
  • Vital Capacity / physiology