Vital capacity (VC), maximal inspiratory pressure (PImax), maximal expiratory pressure (PEmax), peak cough flow (PCF) were measured in succession 8.5 ± 4.3 times (range 4-20 times) for over 20.0 ± 18.7 months (range 2.3-70.3 months) in thirty five patients (average age 59.4 ± 12.1 y.o.) with amyotrophic lateral sclerosis (ALS). All measured values declined with progress of the disease.
Mean rate of change per month (MRC/mo) for percent-predicted VC (VC%) correlated with MRC/mo for PImax (r = 0.840, p < 0.0001), PEmax (r = 0.721, p = 0.0002) and PCF (r = 0.666, p = 0.0025).
Eleven patients were offered non-invasive ventilation (NIV) and eight patients were offered tracheal ventilation (TV). MRC/mo for VC% in spontaneous breathing period was slightly greater in the absolute value than that in NIV period. The declining rate of VC% seemed to be eased by NIV.
Fourteen patients with decreased VC% below 25% in spontaneous period were divided into four groups as follows.
Group A: VC% decreased rapidly in 1 to 2 years after onset (n = 3, MRC/mo for VC% = -18.32%).
Group B: VC% decreased almost linearly, but not so rapidly as group A (n = 7, MRC/mo for VC% = 5.99%).
Group C: VC% maintained about 50% in 4 to 5 years after onset, and rapidly decreased after that stable period ( n = 2, stable period MRC/mo for VC% = 0.07%, declining period MRC/mo for VC% = -11.66%).
Group D: VC% maintained 25% or more after onset over 8 years and declined slowly (n = 2, MRC/mo for VC% = -1.95%).
The results indicate that VC is the most important measurement for periodical evaluation and that declining patterns of VC% may be useful to expect respiratory dysfunction.
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