Amyotrophic lateral sclerosis

EL Feldman, SA Goutman, S Petri, L Mazzini… - The Lancet, 2022 - thelancet.com
Amyotrophic lateral sclerosis is a fatal CNS neurodegenerative disease. Despite intensive
research, current management of amyotrophic lateral sclerosis remains suboptimal from …

Amyotrophic lateral sclerosis: a clinical review

P Masrori, P Van Damme - European journal of neurology, 2020 - Wiley Online Library
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder affecting primarily the
motor system, but in which extra‐motor manifestations are increasingly recognized. The loss …

Emerging insights into the complex genetics and pathophysiology of amyotrophic lateral sclerosis

SA Goutman, O Hardiman, A Al-Chalabi… - The Lancet …, 2022 - thelancet.com
Amyotrophic lateral sclerosis is a fatal neurodegenerative disease. The discovery of genes
associated with amyotrophic lateral sclerosis, commencing with SOD1 in 1993, started fairly …

[PDF][PDF] Phase separation and neurodegenerative diseases: a disturbance in the force

A Zbinden, M Pérez-Berlanga, P De Rossi… - Developmental cell, 2020 - cell.com
Protein aggregation is the main hallmark of neurodegenerative diseases. Many proteins
found in pathological inclusions are known to undergo liquid-liquid phase separation, a …

TDP-43 proteinopathies: a new wave of neurodegenerative diseases

EMJ De Boer, VK Orie, T Williams, MR Baker… - Journal of Neurology …, 2021 - jnnp.bmj.com
Inclusions of pathogenic deposits containing TAR DNA-binding protein 43 (TDP-43) are
evident in the brain and spinal cord of patients that present across a spectrum of …

[PDF][PDF] Phase separation of FUS is suppressed by its nuclear import receptor and arginine methylation

M Hofweber, S Hutten, B Bourgeois, E Spreitzer… - Cell, 2018 - cell.com
Cytoplasmic FUS aggregates are a pathological hallmark in a subset of patients with
frontotemporal dementia (FTD) or amyotrophic lateral sclerosis (ALS). A key step that is …

Clinical neurology and epidemiology of the major neurodegenerative diseases

MG Erkkinen, MO Kim… - Cold Spring Harbor …, 2018 - cshperspectives.cshlp.org
Neurodegenerative diseases are a common cause of morbidity and cognitive impairment in
older adults. Most clinicians who care for the elderly are not trained to diagnose these …

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 …

Frontotemporal dementia

NT Olney, S Spina, BL Miller - Neurologic clinics, 2017 - neurologic.theclinics.com
Frontotemporal dementia (FTD) has undergone numerous changes in nomenclature and
categorization schemes since it was first described by Pick in 1892. Presently, FTD …

[HTML][HTML] The overlapping genetics of amyotrophic lateral sclerosis and frontotemporal dementia

YA Abramzon, P Fratta, BJ Traynor… - Frontiers in neuroscience, 2020 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are two diseases
that form a broad neurodegenerative continuum. Considerable effort has been made to …