[Design and validation of a classification system for assessing the degree of disability of patients with amyotrophic lateral sclerosis].

INTRODUCTION

Currently there is no system of classification validated and commonly used to allow staging the severity of amyotrophic lateral sclerosis.

PATIENTS AND

METHODS

Based on the ALSFRS-R, four domains were established (bulbar, manual skills, gross motor function and respiratory function). Each item had a 0 score if the rate was >= 3 (independence) or 1 if the rate was < 3 (dependence). The functional classification scale was defined as follows: stage 1, without a loss of independence on any domain, until stage 5, with loss of independence in all four domains. This classification is correlated with the need of external support, quality of life with the application of the ALSAQ-40 scale, muscle strength and survival.

RESULTS

From a total of 244 patients, 14.3% were in stage 1, 23.8% in stage 2, 21.3% in stage 3, 19.3% in stage 4 and 21.3% in stage 5. Muscle strength and quality of life were inversely proportional to the stage of the disease, while the need for external aids is directly related to the increase in disease stages 1 to 5 (p < 0.012). Increased survival of patients was found in stages 1 and 2 with respect to those in stages 3, 4 and 5 (p = 0.004).

CONCLUSION

The proposed classification system is easy to apply and correlates well with the patient's clinical, quality of life, resource requirements and survival.