Journal
AMYOTROPHIC LATERAL SCLEROSIS AND FRONTOTEMPORAL DEGENERATION
Volume 18, Issue 3-4, Pages 153-174Publisher
TAYLOR & FRANCIS LTD
DOI: 10.1080/21678421.2016.1267768
Keywords
Amyotrophic lateral sclerosis; frontotemporal dementia; neuropsychology; cognition; behaviour; genetics
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Funding
- ALS Society of Canada
- Michael Halls Endowment
- Windsor-Essex County ALS Society
- National Institute for Health Research (NIHR) Dementia Biomedical Research Unit at South London
- NCATS [U54 NS092091]
- NINDS [U54 NS092091]
- Maudsley NHS Foundation Trust and King's College London
- MRC [MR/K01014X/1] Funding Source: UKRI
- Medical Research Council [MR/K01014X/1, MR/K026992/1] Funding Source: researchfish
- Motor Neurone Disease Association [Turner/Jan13/944-795] Funding Source: researchfish
- NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE [U54NS092091] Funding Source: NIH RePORTER
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This article presents the revised consensus criteria for the diagnosis of frontotemporal dysfunction in amyotrophic lateral sclerosis (ALS) based on an international research workshop on frontotemporal dementia (FTD) and ALS held in London, Canada in June 2015. Since the publication of the Strong criteria, there have been considerable advances in the understanding of the neuropsychological profile of patients with ALS. Not only is the breadth and depth of neuropsychological findings broader than previously recognised - including deficits in social cognition and language - but mixed deficits may also occur. Evidence now shows that the neuropsychological deficits in ALS are extremely heterogeneous, affecting over 50% of persons with ALS. When present, these deficits significantly and adversely impact patient survival. It is the recognition of this clinical heterogeneity in association with neuroimaging, genetic and neuropathological advances that has led to the current re-conceptualisation that neuropsychological deficits in ALS fall along a spectrum. These revised consensus criteria expand upon those of 2009 and embrace the concept of the frontotemporal spectrum disorder of ALS (ALS-FTSD).
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