The ‘neurophysiological index’ predicts survival in amyotrophic lateral sclerosis

• Autor(es): Swash, Michael ; Carvalho, Mamede
• Data: 2019
• Identificador Persistente: http://hdl.handle.net/10451/49100
• Origem: Repositório da Universidade de Lisboa
• Tipo de Documento: Artigo científico
• Idioma: Inglês
• Contribuidor(es): Repositório Científico de Acesso Aberto da ULisboa

Descrição

Planning care in amyotrophic lateral sclerosis (ALS), as well as designing clinical trials of putative therapies, requires measurement of disease severity, and rate of change. For some such purposes an overall measure of disease severity is required, such as the amyotrophic lateral sclerosis functional rating scale (ALS-FRS) which takes account of various domains of disease-related difficulty, including dressing, washing, taking food, swallowing, standing and walking, breathing and coughing. However, for testing a response to a therapeutic a measurement sensitive to the disease process is needed. This need not be an overall measure but could be quite focused. Measurements of strength, whether using a hand-held dynamometer, or a clinical rating scale such as the MRC scale have been deployed, but there is a wide range of variance in such measures, and their inter-rater variability is suspect. In clinical practice, measures of ventilatory capacity are particularly important, but patient cooperation and marked facial weakness often undermine their sensitivity.