Respiratory phenotypes in amyotrophic lateral sclerosis as determined by respiratory questions on the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised and their relation to respiratory tests.

Susana Pinto,Miguel Oliveira Santos,Marta Gromicho,Michael Swash,Mamede de Carvalho

European journal of neurology（2023）

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摘要

These three distinct ALS phenotypic respiratory categories represent progressive stages of ventilatory dysfunction, supporting ALSFRS-R clinical relevance. Orthopnoea is a severe symptom that should prompt NIV, phrenic nerve response being an independent predictor. Early NIV promotes similar survival for G2 and G3.

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关键词

amyotrophic lateral sclerosis,nocturnal pulsed oximetry,phrenic nerve,respiratory function,respiratory tests

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