Skip to main content Accessibility help

We use cookies to distinguish you from other users and to provide you with a better experience on our websites. Close this message to accept cookies or find out how to manage your cookie settings.

Close cookie message
×
Home
Hostname: page-component-ffbbcc459-slw7l Total loading time: 0.167 Render date: 2022-03-06T06:46:42.209Z Has data issue: true Feature Flags: { "shouldUseShareProductTool": true, "shouldUseHypothesis": true, "isUnsiloEnabled": true, "useRatesEcommerce": false, "useNewApi": true }
  • English
International Psychogeriatrics International Psychogeriatrics

Article contents

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.
Get access Rights & Permissions[Opens in a new window]

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 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Chare, L. et al. (2014). New criteria for frontotemporal dementia syndromes: clinical and pathological diagnostic implications. Journal of Neurology, Neurosurgery and Psychiatry, 85, 865–870.CrossRefGoogle ScholarPubMed
Josephs, K. A. et al. (2010). Caudate atrophy on MRI is a characteristic feature of FTLD‐FUS. European Journal of Neurology, 17, 969–975.CrossRefGoogle ScholarPubMed
Kwiatkowski, T. J. et al. (2009). Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis. Science, 323, 1205–1208.CrossRefGoogle ScholarPubMed
Munoz, D. G. et al. (2009). FUS pathology in basophilic inclusion body disease. Acta Neuropathologica, 118, 617–627.CrossRefGoogle ScholarPubMed
Neumann, M., Rademakers, R., Roeber, S., Baker, M., Kretzschmar, H. A. and Mackenzie, I. R. (2009). A new subtype of frontotemporal lobar degeneration with FUS pathology. Brain, 132, 2922–2931.CrossRefGoogle ScholarPubMed
Rascovsky, K. et al. (2011). Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain, 134, 2456–2477.CrossRefGoogle ScholarPubMed
Rohrer, J. D. et al. (2010). The clinical and neuroanatomical phenotype of FUS associated frontotemporal lobar degeneration. Journal of Neurology, Neurosurgery and Psychiatry, 82, 1405–1407.CrossRefGoogle ScholarPubMed
Seelaar, H. et al. (2010). Frequency of ubiquitin and FUS-positive, TDP-43-negative frontotemporal lobar degeneration. Journal of Neurology, 257, 747–753.CrossRefGoogle ScholarPubMed
Snowden, J. S. et al. (2011). The most common type of FTLD-FUS (aFTLD-U) is associated with a distinct clinical form of frontotemporal dementia but is not related to mutations in the FUS gene. Acta Neuropathologica, 122, 99–110.CrossRefGoogle Scholar
Urwin, H. et al. (2010). FUS pathology defines the majority of tau-and TDP-43-negative frontotemporal lobar degeneration. Acta Neuropathologica, 120, 33–41.CrossRefGoogle ScholarPubMed
4
Cited by

Send article to Kindle

To send this article to your Kindle, first ensure no-reply@cambridge.org is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about sending to your Kindle. Find out more about sending to your Kindle.

Note you can select to send to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be sent to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

Find out more about the Kindle Personal Document Service.

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
Available formats
×

Send article to Dropbox

To send this article to your Dropbox account, please select one or more formats and confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your <service> account. Find out more about sending content to Dropbox.

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
Available formats
×

Send article to Google Drive

To send this article to your Google Drive account, please select one or more formats and confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your <service> account. Find out more about sending content to Google Drive.

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
Available formats
×
×

Reply to: Submit a response

Please enter your response.

Your details

Please enter a valid email address.

Conflicting interests

Do you have any conflicting interests? *