Abstract
Background: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that is ultimately fatal but characterized by substantial phenotypic heterogeneity, which is known to impact long-term course and survival. This study investigated clinical determinants of disease progression and outcome in a large cohort of patients with ALS. Methods: Retrospective analysis included comprehensive data from 625 patients who attended a tertiary ALS centre at least twice. Patients were stratified according to five distinct clinical phenotypes: classical ALS; bulbar ALS; ALS with frontotemporal dementia (ALS-FTD); upper motor neuron predominant (UMNP); and lower motor neuron predominant (LMNP). Results: This study confirmed higher age at symptom onset, shorter latency to diagnosis and more rapid decline in the revised ALS Functional Rating Scale sum score as predictors of poor prognosis. Hazard ratios for shorter survival were higher in patients with ALS-FTD versus classical ALS, and in patients with versus without chronic obstructive pulmonary disease (COPD). Mean survival was longest in the UMNP phenotype group. Conclusions: This study confirmed established predictors of shorter survival in ALS and showed that concomitant COPD in particular relates to poor outcome.
Highlights
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by involvement of both upper and lower motor neurons [1]
The findings suggest that higher age at symptom onset, faster disease progression following disease manifestation, shorter delay to diagnosis and concomitant chronic obstructive pulmonary disease (COPD) predict shorter survival
It seems obvious that pre-existing lung disease puts patients at particular risk for early ventilatory failure when diaphragm weakness evolves [8], our observation highlights that patients with both ALS and COPD require increased monitoring for respiratory impairment
Summary
Accepted: 9 April 2021Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by involvement of both upper and lower motor neurons [1]. Neurodegeneration may affect the pyramidal tract or the anterior horn cells on different bulbar and spinal levels, resulting in a variety of clinical phenotypes. Both spasticity and hyperreflexia may be present in conjunction with muscle fasciculations, atrophy and weakness, affecting most skeletal muscles [1,2]. Median survival has been reported to be 2.5–5 years after symptom onset with substantial variability between different studies [3,4,5,6,7]. Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that is fatal but characterized by substantial phenotypic heterogeneity, which is known to impact long-term course and survival. Hazard ratios for shorter survival were higher in patients with
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