Journal
AMYOTROPHIC LATERAL SCLEROSIS AND FRONTOTEMPORAL DEGENERATION
Volume 24, Issue 3-4, Pages 165-174Publisher
TAYLOR & FRANCIS LTD
DOI: 10.1080/21678421.2022.2150555
Keywords
ALS; diagnosis; prognosis; clinical stage; clinical trials
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Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder with difficulties in diagnosis and prognosis assessment. This article provides a clinical overview of balancing evidence-based medicine and patient needs, as well as explores factors to improve diagnostic efficiency and assessment of disease progression.
Amyotrophic lateral sclerosis (ALS) is a rare, progressive multi-system neurodegenerative disorder. Its clinical presentation varies considerably leading to delays in diagnosis, which has dire consequences in a disease where early intervention is key to optimize outcomes and limit care giver burden. There are a range of diagnostic criteria available to aid ALS diagnosis, as well staging methods to assess disease progression. However, they all suffer from inter-rater variability, complexity, and confusion in use. Such difficulties, when medical appointment times are limited and becoming more virtually based, have the potential to amplify uncertainty and errors in ALS diagnosis and prognosis. This review provides a clinical overview of the best way to balance the needs of evidence-based medicine and the patient. We focus on ALS diagnostic criteria and staging systems currently in use in clinical practice and explore factors that could enhance diagnostic efficiency and assessment of disease progression.
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