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Rapidly progressive Fronto-temporal dementia (FTD) associated with Frontotemporal lobar degeneration (FTLD) in the presence of Fused in Sarcoma (FUS) protein: a rare, sporadic, and aggressive form of FTD

Published online by Cambridge University Press:  29 June 2017

Nicholas I. Bradfield Open the ORCID record for Nicholas I. Bradfield [Opens in a new window] ,
Catriona McLean ,
John Drago ,
David G. Darby  and
David Ames
Nicholas I. Bradfield*
Affiliation:
University of Melbourne, St Vincent's Clinical School, St Vincent's Hospital, Fitzroy, Victoria, Australia
Catriona McLean
Affiliation:
Alfred Health, Prahran, Victoria, Australia The Florey Institute of Neurosciences and Mental Health, Parkville, Victoria, Australia
John Drago
Affiliation:
The Florey Institute of Neurosciences and Mental Health, Parkville, Victoria, Australia St Vincent's Hospital, Fitzroy, Victoria, Australia
David G. Darby
Affiliation:
The Florey Institute of Neurosciences and Mental Health, Parkville, Victoria, Australia
David Ames
Affiliation:
The Florey Institute of Neurosciences and Mental Health, Parkville, Victoria, Australia University of Melbourne Academic Unit for Psychiatry of Old Age, St George's Hospital, Kew, Victoria, Australia National Ageing Research Institute, Parkville, Victoria, Australia
*
Correspondence should be addressed to: Nicholas I. Bradfield, University of Melbourne, St Vincent's Clinical School, St Vincent's Hospital, Fitzroy, Victoria, Australia. Phone: +61 411 270 386. Email: n.bradfield@neuropsychology.org.au.
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Abstract

Fronto-temporal dementia (FTD) associated with Fused in Sarcoma (FUS) protein accumulation is an uncommon cause of FTD with a distinct syndrome of young age onset behavioral variant FTD, without a family history of FTD and caudate atrophy. We present a sporadic case of a 61-year-old patient with mixed features of both behavioral variant FTD with later semantic language dissolution associated with pathologically proven FUS. He was older than usual for FUS pathology, his course was rapidly progressive, and he had atypical language features. This case broadens the clinical spectrum caused by FUS-protein-related FTD.

Type
Case Report
Information
International Psychogeriatrics , Volume 29 , Issue 10 , October 2017 , pp. 1743 - 1746
Copyright
Copyright © International Psychogeriatric Association 2017 

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