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 …

Innate immune activation in neurodegenerative disease

MT Heneka, MP Kummer, E Latz - Nature Reviews Immunology, 2014 - nature.com
The triggering of innate immune mechanisms is emerging as a crucial component of major
neurodegenerative diseases. Microglia and other cell types in the brain can be activated in …

Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6

C Vance, B Rogelj, T Hortobágyi, KJ De Vos… - Science, 2009 - science.org
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that is familial in
10% of cases. We have identified a missense mutation in the gene encoding fused in …

Controversies and priorities in amyotrophic lateral sclerosis

MR Turner, O Hardiman, M Benatar, BR Brooks… - The Lancet …, 2013 - thelancet.com
Two decades after the discovery that 20% of familial amyotrophic lateral sclerosis (ALS)
cases were linked to mutations in the superoxide dismutase-1 (SOD1) gene, a substantial …

TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis

T Arai, M Hasegawa, H Akiyama, K Ikeda… - Biochemical and …, 2006 - Elsevier
Ubiquitin-positive tau-negative neuronal cytoplasmic inclusions and dystrophic neurites are
common pathological features in frontotemporal lobar degeneration (FTLD) with or without …

TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis

J Sreedharan, IP Blair, VB Tripathi, X Hu, C Vance… - Science, 2008 - science.org
Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disorder characterized
pathologically by ubiquitinated TAR DNA binding protein (TDP-43) inclusions. The function …

Amyotrophic lateral sclerosis

LP Rowland, NA Shneider - New England Journal of Medicine, 2001 - Mass Medical Soc
Charcot described amyotrophic lateral sclerosis (ALS) in 1874. Despite progress, this
creeping paralysis, known colloquially as Lou Gehrig's disease, is still not visibly affected by …

Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis

L Ferraiuolo, J Kirby, AJ Grierson, M Sendtner… - Nature Reviews …, 2011 - nature.com
Amyotrophic lateral sclerosis (ALS) is a genetically diverse disease. At least 15 ALS-
associated gene loci have so far been identified, and the causative gene is known in …

ALS: a disease of motor neurons and their nonneuronal neighbors

S Boillée, CV Velde, DW Cleveland - Neuron, 2006 - cell.com
Amyotrophic lateral sclerosis is a late-onset progressive neurodegenerative disease
affecting motor neurons. The etiology of most ALS cases remains unknown, but 2% of …