The authors have shown in a recent paper that survival with amyotrophic lat
eral sclerosis (ALS) can be increased by the use of non-invasive methods of
assisted ventilation (Bipap(R)). However, the progression of muscle weakne
ss was not affected and the quality of life was not positively enhanced. In
ALS, reduced physical activity may partially be secondary to alveolar hypo
ventilation syndrome. This leads to deconditioning of ALS/motor neuron dise
ase (ALS/MND) patients. The authors decided to investigate the possibility
of reducing motor decline by exercising these patients to the anaerobic thr
eshold, but simultaneously compensating the respiratory insufficiency with
the Bipap STD(R). We conducted a controlled single blind study, exercising
eight consecutive ALS/MND patients and used a control group of 12 ALS/MND p
atients. The patients were all evaluated during a 1 year period. Respirator
y function tests (RFT) were performed at entry and then at 6 month interval
s. Barthel, Functional Independent Mobility scale (FIM) and Spinal and Bulb
ar Norris scores were recorded every 3 months. There was a significant diff
erence between the two groups with respect to FIM scores (P < 0.03), but no
t Barthel scores (P < 0.8). A slower clinical course (Spinal Norris score P
< 0.02) and a significant difference in the slope of the RFT (P < 0.008) w
ere observed in the treated group, suggesting that exercise may be benefici
al in ALS patients once Bipap(R) is used to control peripheral and muscle o
xygenation. (C) 1999 Elsevier Science BN. All rights reserved.