The results of this study prove that the Spanish adaptation of the ECAS scale preserves the internal consistency and construct validity of its original version.

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Background: ALS is a neurodegenerative disease of the entire motor system that most frequently ends with respiratory arrest in few years. Its diagnosis and the rapid progression of the motor dysfunctions produce a continued emotional impact. Studies on this impact are helpful to plan adequate psychotherapeutic strategies. Objective: To assess and analyze: First: How the patients with ALS perceive their emotional health. Second: The emotional impact of their physical disabilities. Third: The physical disabilities with highest emotional impact. Fourth: The feelings with highest emotional impact. Methods: Up to 110 Spanish patients with ALS were assessed less than 1 year from diagnosis, then twice more at 6 month intervals, using the ALS Quality of Life Assessment Questionnaire (ALSAQ-40) validated for use in Spanish. Descriptive analysis and correlation between variables were obtained. Results: Worries about the future, of lack of freedom, and of being a burden were prevalent feelings. On average depression was felt only “sometimes.” Only 25% of the variations in the emotional state were explained by changes in the physical state at first evaluation, and 16% at the last one. Emotional functioning correlated significantly with the physical disabilities at first and second evaluation, less so at third. Communication disabilities always had the highest impact. Depression at first evaluation and hopelessness at the next two evaluations had the highest emotional impact. Hopelessness did not correlate with any physical disability at the third evaluation. On the whole, emotional dysfunction was self perceived as intermediate (between none and worst), and remained stable at 1 year follow up, in both bulbar and spinal onset patients. Conclusions: Physical dysfunctions per se have a limited role in patients´ emotional distress. Communication disabilities, as well as feelings of depression at early stages of illness, and of hopelessness later on, had the most impact. This requires their careful therapeutic attention. On average, Spanish patients with ALS cope with their disease, overcoming depression, which is not felt often, and with just mid levels of emotional dysfunction.

ALSFRS-R is a tool designed to measure disease progress in ALS patients. It consists of 12 items grouped into four functions designed to assess disabilities according to the Activities of daily living (ADL). Our objective was to validate the Spanish version of ALSFRS-R based on the original version. Four examiners assessed 73 ALS patients, applying the ALSFRS-R, ALSAQ-40 and the respiratory function variable assessed by the SRI scale, which measures respiratory insufficiency. Internal consistency and test-retest correlations were measured using Cronbach's alpha and Spearman's Rho tests. Factor analysis was performed by applying Varimax rotation and Kaiser standardization. Validity was analysed based on correlations between items in the ALSFRS-R scales and equivalents in the ALSAQ-40 and SRI questionnaires. The results showed high internal consistency (0.77-0.95) and a good test-retest correlation (0.80-0.95). Factor analysis showed a 73.3% principal component contribution; the weight of each item regarding their corresponding factors was 0.7-0.9. High correlations were observed (rs >0.60) between corresponding factors of ALSFRS-R/ALSAQ-40 and ALSFRS-R/SRI. We conclude that the version obtained from the ALSFRS-R maintains the internal consistency and validity of the construct of the original scale. The Spanish version of ALSFRS-R is available for readers at http://www.fundela.es/verOtras.php.