Susana Pinto1, Ruth Geraldes2, Nuno Vaz1, Anabela Pinto3, Mamede de Carvalho4. 1. Neuromuscular Unit, Institute of Molecular Medicine - Faculty of Medicine, University of Lisbon, Lisbon, Portugal. 2. Department of Neurosciences, Hospital de Santa Maria, Lisbon, Portugal. 3. Neuromuscular Unit, Institute of Molecular Medicine - Faculty of Medicine, University of Lisbon, Lisbon, Portugal; Department of Physical Medicine and Rehabilitation, Hospital de Santa Maria, Lisbon, Portugal. 4. Neuromuscular Unit, Institute of Molecular Medicine - Faculty of Medicine, University of Lisbon, Lisbon, Portugal; Department of Neurosciences, Hospital de Santa Maria, Lisbon, Portugal. Electronic address: mamedemg@mail.telepac.pt.
Abstract
OBJECTIVE: Phrenic nerve motor amplitude (Diaphr Ampl) is predictive of hypoventilation in amyotrophic lateral sclerosis (ALS). We aimed to evaluate its change over disease course and to correlate it to other measurements. METHODS: Forty-nine unselected patients (35 men, 13 bulbar-onset, 56.5+/-8.9 years) with definitive or probable ALS were included. They were evaluated at entry (time 0) and 4-6 months (5.2+/-1.0) later (time 1) with: functional ALS rating scale (ALS-FRS) and respiratory subscore (ALS-FRSr); forced vital capacity (FVC); maximal inspiratory pressure (MIP); mean O(2) saturation overnight (SpO(2)mean); sniff maximal inspiratory pressure (SNIP); Diaphr Ampl and mean amplitude of the ulnar nerve response (ADM Ampl). RESULTS: ALS-FRS, ALS-FRSr, Diaphr Ampl, FVC, SNIP, ADM Ampl (p<0.01) and SpO(2)mean (p<0.05) declined significantly. MIP did not change significantly (p=0.203). Coefficient of variation was similar for FVC, Diaphr Ampl, ADM Ampl and ALS-FRS but higher for SNIP. The percentage of change for Diaphr Ampl was significantly correlated to FVC and SNIP, but not to ADM Ampl or ALS-FRS. CONCLUSIONS: Diaphr Ampl decreased significantly in a short period of time and its change is correlated to other respiratory tests. This test can be useful in patients with marked facial weakness or uncooperative. SIGNIFICANCE: Diaphr Ampl is useful to monitor respiratory function in ALS patients and can be applied in clinical trials.
OBJECTIVE: Phrenic nerve motor amplitude (Diaphr Ampl) is predictive of hypoventilation in amyotrophic lateral sclerosis (ALS). We aimed to evaluate its change over disease course and to correlate it to other measurements. METHODS: Forty-nine unselected patients (35 men, 13 bulbar-onset, 56.5+/-8.9 years) with definitive or probable ALS were included. They were evaluated at entry (time 0) and 4-6 months (5.2+/-1.0) later (time 1) with: functional ALS rating scale (ALS-FRS) and respiratory subscore (ALS-FRSr); forced vital capacity (FVC); maximal inspiratory pressure (MIP); mean O(2) saturation overnight (SpO(2)mean); sniff maximal inspiratory pressure (SNIP); Diaphr Ampl and mean amplitude of the ulnar nerve response (ADM Ampl). RESULTS:ALS-FRS, ALS-FRSr, Diaphr Ampl, FVC, SNIP, ADM Ampl (p<0.01) and SpO(2)mean (p<0.05) declined significantly. MIP did not change significantly (p=0.203). Coefficient of variation was similar for FVC, Diaphr Ampl, ADM Ampl and ALS-FRS but higher for SNIP. The percentage of change for Diaphr Ampl was significantly correlated to FVC and SNIP, but not to ADM Ampl or ALS-FRS. CONCLUSIONS: Diaphr Ampl decreased significantly in a short period of time and its change is correlated to other respiratory tests. This test can be useful in patients with marked facial weakness or uncooperative. SIGNIFICANCE: Diaphr Ampl is useful to monitor respiratory function in ALS patients and can be applied in clinical trials.
Authors: Nicole L Nichols; J Van Dyke; L Nashold; I Satriotomo; M Suzuki; G S Mitchell Journal: Respir Physiol Neurobiol Date: 2013-05-18 Impact factor: 1.931
Authors: Jose C Roche; Ricardo Rojas-Garcia; Kirsten M Scott; William Scotton; Catherine E Ellis; Rachel Burman; Lokesh Wijesekera; Martin R Turner; P Nigel Leigh; Christopher E Shaw; Ammar Al-Chalabi Journal: Brain Date: 2012-01-23 Impact factor: 13.501