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 …

[HTML][HTML] Amyotrophic lateral sclerosis

MC Kiernan, S Vucic, BC Cheah, MR Turner, A Eisen… - The lancet, 2011 - thelancet.com
Amyotrophic lateral sclerosis (ALS) is an idiopathic, fatal neurodegenerative disease of the
human motor system. In this Seminar, we summarise current concepts about the origin of the …

Neuropathology of amyotrophic lateral sclerosis and its variants

S Saberi, JE Stauffer, DJ Schulte… - Neurologic …, 2015 - neurologic.theclinics.com
The first case reports of amyotrophic lateral sclerosis (ALS) date back to Charles Bell in
1824. 1 Although a variety of other clinical descriptions followed throughout the 1850s, 2–4 …

Neuroimaging signatures of frontotemporal dementia genetics: C9ORF72, tau, progranulin and sporadics

JL Whitwell, SD Weigand, BF Boeve, ML Senjem… - Brain, 2012 - academic.oup.com
A major recent discovery was the identification of an expansion of a non-coding GGGGCC
hexanucleotide repeat in the C9ORF72 gene in patients with frontotemporal dementia and …

Motor neuron dysfunction in frontotemporal dementia

JR Burrell, MC Kiernan, S Vucic, JR Hodges - Brain, 2011 - academic.oup.com
Frontotemporal dementia and motor neuron disease share clinical, genetic and pathological
characteristics. Motor neuron disease develops in a proportion of patients with …

Frontotemporal dementia

D Neary, J Snowden, D Mann - The Lancet Neurology, 2005 - thelancet.com
Frontotemporal dementia (FTD) is a focal clinical syndrome characterised by profound
changes in personality and social conduct and associated with circumscribed degeneration …

From animal models to human disease: a genetic approach for personalized medicine in ALS

V Picher-Martel, PN Valdmanis, PV Gould… - Acta neuropathologica …, 2016 - Springer
Abstract Amyotrophic Lateral Sclerosis (ALS) is the most frequent motor neuron disease in
adults. Classical ALS is characterized by the death of upper and lower motor neurons …

Biomarkers in amyotrophic lateral sclerosis

MR Turner, MC Kiernan, PN Leigh, K Talbot - The Lancet Neurology, 2009 - thelancet.com
Amyotrophic lateral sclerosis (ALS; motor neuron disease) is a relentlessly progressive
disorder. After half a century of trials, only one drug with modest disease-modifying potency …

Corpus callosum involvement is a consistent feature of amyotrophic lateral sclerosis

N Filippini, G Douaud, CE Mackay, S Knight, K Talbot… - Neurology, 2010 - AAN Enterprises
Objective: While the hallmark of amyotrophic lateral sclerosis (ALS) is corticospinal tract in
combination with lower motor neuron degeneration, the clinical involvement of both …

Quantifying disease progression in amyotrophic lateral sclerosis

NG Simon, MR Turner, S Vucic… - Annals of …, 2014 - Wiley Online Library
Amyotrophic lateral sclerosis (ALS) exhibits characteristic variability of onset and rate of
disease progression, with inherent clinical heterogeneity making disease quantitation …