ArticleJournal of clinical medicine2021
Clinical Determinants of Disease Progression in Amyotrophic Lateral Sclerosis-A Retrospective Cohort Study.
Article in Journal of clinical medicine, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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Who cites it
14 citing papers in PubMed, 22 citations in OpenAlex.
- Safety, tolerability, and efficacy of fasudil in amyotrophic lateral sclerosis (ROCK-ALS): a phase 2, randomised, double-blind, placebo-controlled trial.The Lancet. Neurology · 2024Trial
- Identification and validation of a tear fluid-derived protein biomarker signature in patients with amyotrophic lateral sclerosis.Acta neuropathologica communications · 2025Article
- Motor phenotypes of amyotrophic lateral sclerosis - a three-determinant anatomical classification based on the region of onset, propagation of motor symptoms, and the degree of upper and lower motor neuron dysfunction.Neurological research and practice · 2025Article
- Blood DNA Methylation in Nuclear and Mitochondrial Sequences Links to Malnutrition and Poor Prognosis in ALS: A Longitudinal Study.Nutrients · 2025Article
- Targeting mitophagy in neurodegenerative diseases.Nature reviews. Drug discovery · 2025Review
- Quantifying neurodegeneration within subdivisions of core motor pathways in amyotrophic lateral sclerosis using diffusion MRI.Journal of neurology · 2025Article
- Emerging biomarkers in amyotrophic lateral sclerosis: from pathogenesis to clinical applications.Frontiers in molecular biosciences · 2025Review
- Enhancing ALS progression tracking with semi-supervised ALSFRS-R scores estimated from ambient home health monitoring.Frontiers in digital health · 2025Article
- Serum neurofilament light chain in distinct phenotypes of amyotrophic lateral sclerosis: A longitudinal, multicenter study.European journal of neurology · 2024Article
- Temporal stratification of amyotrophic lateral sclerosis patients using disease progression patterns.Nature communications · 2024Article
- Venous blood parameters in determination of respiratory impairment in amyotrophic lateral sclerosis.Scientific reports · 2023Article
- Amyotrophic Lateral Sclerosis: Advances and Prospects.Journal of clinical medicine · 2023Article
- Biochemical parameters as a tool to assess the nutritional status of patients with amyotrophic lateral sclerosis.Frontiers in neurology · 2023Article
- Pain-Related Coping Behavior in ALS: The Interplay between Maladaptive Coping, the Patient's Affective State and Pain.Journal of clinical medicine · 2022Article
Corrections and comments
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Authors and funding
4 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundAmyotrophic 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.
methodsRetrospective 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).
resultsThis 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.
conclusionsThis study confirmed established predictors of shorter survival in ALS and showed that concomitant COPD in particular relates to poor outcome.
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