Amyotrophic lateral sclerosis - frontotemporal spectrum disorder (ALS-FTSD): Revised diagnostic criteria

TY - JOUR

T1 - Amyotrophic lateral sclerosis - frontotemporal spectrum disorder (ALS-FTSD)

T2 - Revised diagnostic criteria

AU - Strong, Michael J.

AU - Abrahams, Sharon

AU - Goldstein, Laura H.

AU - Woolley, Susan

AU - Mclaughlin, Paula

AU - Snowden, Julie

AU - Mioshi, Eneida

AU - Roberts-South, Angie

AU - Benatar, Michael

AU - HortobáGyi, Tibor

AU - Rosenfeld, Jeffrey

AU - Silani, Vincenzo

AU - Ince, Paul G.

AU - Turner, Martin R.

PY - 2017/4/3

Y1 - 2017/4/3

N2 - 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).

AB - 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).

KW - Amyotrophic lateral sclerosis

KW - behaviour

KW - cognition

KW - frontotemporal dementia

KW - genetics

KW - neuropsychology

UR - http://www.scopus.com/inward/record.url?scp=85008392695&partnerID=8YFLogxK

U2 - 10.1080/21678421.2016.1267768

DO - 10.1080/21678421.2016.1267768

M3 - Article

AN - SCOPUS:85008392695

SN - 2167-8421

SP - 1

EP - 22

JO - Amyotrophic lateral sclerosis & frontotemporal degeneration

JF - Amyotrophic lateral sclerosis & frontotemporal degeneration

ER -