Can amyotrophic lateral sclerosis patients with respiratory insufficiency exercise?

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Abstract

The authors have shown in a recent paper that survival with amyotrophic lateral sclerosis (ALS) can be increased by the use of non-invasive methods of assisted ventilation (Bipap®). However, the progression of muscle weakness was not affected and the quality of life was not positively enhanced. In ALS, reduced physical activity may partially be secondary to alveolar hypoventilation syndrome. This leads to deconditioning of ALS/motor neuron disease (ALS/MND) patients. The authors decided to investigate the possibility of reducing motor decline by exercising these patients to the anaerobic threshold, but simultaneously compensating the respiratory insufficiency with the Bipap STD®. We conducted a controlled single blind study, exercising eight consecutive ALS/MND patients and used a control group of 12 ALS/MND patients. The patients were all evaluated during a 1 year period. Respiratory function tests (RFT) were performed at entry and then at 6 month intervals. Barthel, Functional Independent Mobility scale (FIM) and Spinal and Bulbar Norris scores were recorded every 3 months. There was a significant difference between the two groups with respect to FIM scores (P<0.03), but not 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) were observed in the treated group, suggesting that exercise may be beneficial in ALS patients once Bipap® is used to control peripheral and muscle oxygenation.

Introduction

Amyotrophic lateral sclerosis/motor neuron disease (ALS/MND) is a progressive and fatal disease in which the most common terminal event is respiratory insufficiency. Treatment of this terminal phase using various methods of non-invasive ventilation is advised by many different authors [1], [2], [3], and has finally been accepted as standard care for ALS/MND patients [4], [5]. However, all patients progress to quadriplegia with substantially reduced quality of life. Prolonging survival with independence in activities of daily living is certainly a major goal in the rehabilitation setting of ALS/MND patients.

Reduced physical activity may partially be secondary to the hypoventilation syndrome and responsible for the deconditioning of MND/ALS patients. We decided to investigate the possibility of reducing motor decline by exercising these patients to the anaerobic threshold, while at the same time compensating the respiratory insufficiency with the Bipap STD®.

Section snippets

Material and methods

To test the effects of an exercise program on ALS/MND patients, we conducted a prospective, single blind controlled clinical trial of 20 consecutive patients. All patients gave their formal informed consent. Group 1 (treated group) included eight patients, six male and two female, with a mean age of 62±14 years (mean±SD). Group 2 included 12 patients, four female and eight male, aged 64±16 years (mean±SD). All patients were evaluated at their visit following diagnosis and followed-up during 1

Results

Clinical data, including respiratory function parameters, are presented in Table 1, Table 2, Table 3, Table 4, and the respective means, standard deviations and t-tests with the P values are presented in Table 5. Data from Barthel scores and respective slopes are omitted because they were not found to be relevant, in fact we did not find any statistical significance between the two groups during the period of this study (Table 6).

In group 1, at initial observation, two patients (2 and 4) had

Discussion

Non-invasive ventilation in neuromuscular diseases and in MND/ALS patients is a well established technique [9], [10], [11], [12], [13], [14]. There are many advantages of Bipap® over invasive methods: it is less expensive, prolongs survival, and improves quality of life, sleep and exercise tolerance. But some questions about non-invasive ventilation remain; particularly, when should this therapy be started? It is accepted that non-invasive ventilation should be started when its advantages are

Acknowledgements

The authors are indebted to ‘Gasin Co, Medical Division of Portugal’, for cooperating with us with patient assistance, and to Margarida Fernandes for secretarial support.

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