Journal of the neurological sciences
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Clinical Trial
Patients' health-related quality of life and utilities associated with different stages of amyotrophic lateral sclerosis.
Amyotrophic lateral sclerosis (ALS) is a devastating disease that has serious consequences in terms of impairments and disabilities, which are expected to impact on health-related quality of life (HRQL). The aim of the present study was to assess self-reported health status and HRQL, as well as patients' own valuation of their present health state in a sample of patients with different levels of severity of ALS. ⋯ The results from the ALSAQ-40 and EQ-5D descriptive system indicate that patients' HRQL decreases systematically with increasing severity of disease. Patients' mean VAS rating of their own health ranged from 0.74 for stage 1 (early) disease severity, to 0.37 for stage 4 (late stage) disease severity. Utilities elicited via SG were systematically higher than VAS scores and ranged from a mean of 0.79 for stage 1 disease severity to a mean of 0.45 for stage 4 disease severity.
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Multicenter Study
Amyotrophic lateral sclerosis care in Italy: a nationwide study in neurological centers.
Recently, formal standards for the management of amyotrophic lateral sclerosis (ALS) have been proposed by the American Academy of Neurology (AAN). However, there are few information about the actual care of ALS. We have assessed the management of ALS in Italy in various clinical settings, on basis of a self-reported questionnaire. ⋯ Respiratory management seemed to be lacking both in large and in small centers since non-invasive positive pressure ventilation (NIPPV) was proposed by only 70% of large and 50% of small centers; however, the percentage of patients who underwent NIPPV was significantly higher in large centers (p=0.03). Moreover, the discussion of respiratory issues was performed quite late in the course of the disease, usually when the patients have first respiratory symptoms. Therefore, there are considerable opportunities to improve the care of ALS patients in Italy, primarily through the education of neurologists on AAN standards of care for ALS.