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

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 …

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 …

Changes in cognition and behaviour in amyotrophic lateral sclerosis: nature of impairment and implications for assessment

LH Goldstein, S Abrahams - The Lancet Neurology, 2013 - thelancet.com
Increased awareness of cognitive and behavioural change in amyotrophic lateral sclerosis
has been driven by various clinic-based and population-based studies. A frontotemporal …

Behavioural-variant frontotemporal dementia: diagnosis, clinical staging, and management

O Piguet, M Hornberger, E Mioshi… - The Lancet …, 2011 - thelancet.com
Patients with behavioural-variant frontotemporal dementia (bvFTD) present with insidious
changes in personality and interpersonal conduct that indicate progressive disintegration of …

Distinct clinical and pathological characteristics of frontotemporal dementia associated with C9ORF72 mutations

JS Snowden, S Rollinson, JC Thompson, JM Harris… - Brain, 2012 - academic.oup.com
The identification of a hexanucleotide repeat expansion in the C9ORF72 gene as the cause
of chromosome 9-linked frontotemporal dementia and motor neuron disease offers the …

Frontotemporal dementia with the C9ORF72 hexanucleotide repeat expansion: clinical, neuroanatomical and neuropathological features

CJ Mahoney, J Beck, JD Rohrer, T Lashley, K Mok… - Brain, 2012 - academic.oup.com
An expanded hexanucleotide repeat in the C9ORF72 gene has recently been identified as a
major cause of familial frontotemporal lobar degeneration and motor neuron disease …

Neuropathological background of phenotypical variability in frontotemporal dementia

KA Josephs, JR Hodges, JS Snowden… - Acta …, 2011 - Springer
Frontotemporal lobar degeneration (FTLD) is the umbrella term encompassing a
heterogeneous group of pathological disorders. With recent discoveries, the FTLDs have …

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 …