Amyotrophic lateral sclerosis: a neurodegenerative disorder poised for successful therapeutic translation

RJ Mead, N Shan, HJ Reiser, F Marshall… - Nature Reviews Drug …, 2023 - nature.com
Amyotrophic lateral sclerosis (ALS) is a devastating disease caused by degeneration of
motor neurons. As with all major neurodegenerative disorders, development of disease …

Amyotrophic lateral sclerosis

MA Van Es, O Hardiman, A Chio, A Al-Chalabi… - The Lancet, 2017 - thelancet.com
Amyotrophic lateral sclerosis is characterised by the progressive loss of motor neurons in
the brain and spinal cord. This neurodegenerative syndrome shares pathobiological …

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

MJ Strong, S Abrahams, LH Goldstein… - … lateral sclerosis and …, 2017 - Taylor & Francis
This article presents the revised consensus criteria for the diagnosis of frontotemporal
dysfunction in amyotrophic lateral sclerosis (ALS) based on an international research …

Recent advances in the diagnosis and prognosis of amyotrophic lateral sclerosis

SA Goutman, O Hardiman, A Al-Chalabi… - The Lancet …, 2022 - thelancet.com
The diagnosis of amyotrophic lateral sclerosis can be challenging due to its heterogeneity in
clinical presentation and overlap with other neurological disorders. Diagnosis early in the …

The epidemiology of ALS: a conspiracy of genes, environment and time

A Al-Chalabi, O Hardiman - Nature Reviews Neurology, 2013 - nature.com
Amyotrophic lateral sclerosis (ALS) is a relentlessly progressive neurodegenerative disease
of motor neurons, resulting in worsening weakness of voluntary muscles until death from …

Antisense proline-arginine RAN dipeptides linked to C9ORF72-ALS/FTD form toxic nuclear aggregates that initiate in vitro and in vivo neuronal death

X Wen, W Tan, T Westergard, K Krishnamurthy… - Neuron, 2014 - cell.com
Summary Expanded GGGGCC (G4C2) nucleotide repeats within the C9ORF72 gene are the
most common genetic mutation associated with both amyotrophic lateral sclerosis (ALS) and …

The frontotemporal dementia-motor neuron disease continuum

JR Burrell, GM Halliday, JJ Kril, LM Ittner, J Götz… - The Lancet, 2016 - thelancet.com
Early reports of cognitive and behavioural deficits in motor neuron disease might have been
overlooked initially, but the concept of a frontotemporal dementia-motor neuron disease …

Amyotrophic lateral sclerosis

O Hardiman, A Al-Chalabi, A Chio, EM Corr… - Nature reviews Disease …, 2017 - nature.com
Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease, is characterized
by the degeneration of both upper and lower motor neurons, which leads to muscle …

ALS-specific cognitive and behavior changes associated with advancing disease stage in ALS

C Crockford, J Newton, K Lonergan, T Chiwera… - Neurology, 2018 - AAN Enterprises
Objective To elucidate the relationship between disease stage in amyotrophic lateral
sclerosis (ALS), as measured with the King's Clinical Staging System, and cognitive and …

Cognitive impairment across ALS clinical stages in a population-based cohort

A Chiò, C Moglia, A Canosa, U Manera, R Vasta… - Neurology, 2019 - AAN Enterprises
Objective To assess the association of the degree of severity of motor impairment to that of
cognitive impairment in a large cohort of patients with amyotrophic lateral sclerosis (ALS) …