The frontotemporal dementia-motor neuron disease continuum

Burrell, James R., Halliday, Glenda M., Kril, Jillian J., Ittner, Lars M., Gotz, Jurgen, Kiernan, Matthew C. and Hodges, John R. (2016) The frontotemporal dementia-motor neuron disease continuum. The Lancet, 388 10047: 919-931. doi:10.1016/S0140-6736(16)00737-6

Author Burrell, James R.
Halliday, Glenda M.
Kril, Jillian J.
Ittner, Lars M.
Gotz, Jurgen
Kiernan, Matthew C.
Hodges, John R.
Title The frontotemporal dementia-motor neuron disease continuum
Journal name The Lancet   Check publisher's open access policy
ISSN 1474-547X
Publication date 2016-03-14
Year available 2016
Sub-type Critical review of research, literature review, critical commentary
DOI 10.1016/S0140-6736(16)00737-6
Open Access Status Not Open Access
Volume 388
Issue 10047
Start page 919
End page 931
Total pages 13
Place of publication London, United Kingdom
Publisher Lancet Publishing Group
Language eng
Formatted abstract
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 continuum has emerged during the past decade. Frontotemporal dementia-motor neuron disease is now recognised as an important dementia syndrome, which presents substantial challenges for diagnosis and management. Frontotemporal dementia, motor neuron disease, and frontotemporal dementia-motor neuron disease are characterised by overlapping patterns of TAR DNA binding protein (TDP-43) pathology, while the chromosome 9 open reading frame 72 (C9orf72) repeat expansion is common across the disease spectrum. Indeed, the C9orf72 repeat expansion provides important clues to disease pathogenesis and suggests potential therapeutic targets. Variable diagnostic criteria identify motor, cognitive, and behavioural deficits, but further refinement is needed to define the clinical syndromes encountered in frontotemporal dementia-motor neuron disease.
Keyword Medicine, General & Internal
General & Internal Medicine
Q-Index Code C1
Q-Index Status Provisional Code
Grant ID 1037746
Institutional Status UQ

Document type: Journal Article
Sub-type: Critical review of research, literature review, critical commentary
Collections: HERDC Pre-Audit
Queensland Brain Institute Publications
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Citation counts: TR Web of Science Citation Count  Cited 24 times in Thomson Reuters Web of Science Article | Citations
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