Skip Navigation


Brain Advance Access originally published online on September 30, 2004
Brain 2004 127(11):2441-2451; doi:10.1093/brain/awh265
This Article
Right arrow Full Text Freely available
Right arrow FREE Full Text (PDF) Freely available
Right arrow All Versions of this Article:
127/11/2441    most recent
awh265v1
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in ISI Web of Science
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My Personal Archive
Right arrow Download to citation manager
Right arrow Search for citing articles in:
ISI Web of Science (18)
Right arrowRequest Permissions
Right arrow Disclaimer
Google Scholar
Right arrow Articles by Paviour, D. C.
Right arrow Articles by Holton, J. L.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Paviour, D. C.
Right arrow Articles by Holton, J. L.
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?

Brain Vol. 127 No. 11 © Guarantors of Brain 2004; all rights reserved

Frontotemporal lobar degeneration with ubiquitin-only-immunoreactive neuronal changes: broadening the clinical picture to include progressive supranuclear palsy

D. C. Paviour1,2, A. J. Lees1,3, K. A. Josephs2,4,6, T. Ozawa6, M. Ganguly6, C. Strand6, A. Godbolt2, R. S. Howard5, T. Revesz6 and J. L. Holton6

1 The Sara Koe PSP Research Centre and 2 Dementia Research Centre (UCL), Institute of Neurology, 3 The Reta Lila Weston Institute of Neurological Sciences, London, UK, 4 Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA, 5 National Hospital for Neurology and Neurosurgery and 6 Queen Square Brain Bank and Department of Molecular Neuroscience, Institute of Neurology, UCL, London, UK

Correspondence to: Dr J. L. Holton, Department of Molecular Neuroscience and Queen Square Brain Bank, Institute of Neurology, Queen Square, London WC1N 3BG UK E-mail: j.holton{at}ion.ucl.ac.uk

The frontotemporal lobar degenerations (FTLDs) are a group of disorders in which the clinical picture is not necessarily predictive of the underlying neuropathology. The FTLD with ubiquitin-only-immunoreactive neuronal changes (FTLD-U) subtype is pathologically characterized by ubiquitin-positive, tau and {alpha}-synuclein-negative neuronal cytoplasmic inclusions in the frontotemporal cortex and hippocampal dentate fascia. When similar pathological changes are accompanied by histological features of motor neuron disease (MND), the term FTLD-MND is used. The latter pathological changes may be found in patients with or without clinical evidence of MND. We retrospectively reviewed the clinical details of three patients with a rapidly progressive, levodopa-unresponsive bradykinetic-rigid syndrome and frontal cognitive impairment. A diagnosis of progressive supranuclear palsy (PSP) had been considered in all three cases at initial presentation. Two of the cases fulfilled clinical diagnostic criteria for PSP, which was the final clinical diagnosis during life. Pathological analysis showed typical histological appearances of FTLD-MND in two cases and of FTLD-U in one case. Semi-quantitative analysis of pathological load seemed to correlate with the clinical phenotype. FTLD-U or FTLD-MND should be considered in the differential diagnosis of progressive frontal dementia with an akinetic rigid syndrome and supranuclear gaze palsy or Steele–Richardson–Olszewski disease.


Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us    What's this?


This article has been cited by other articles:


Home page
 BrainHome page
S. Garbutt, A. Matlin, J. Hellmuth, A. K. Schenk, J. K. Johnson, H. Rosen, D. Dean, J. Kramer, J. Neuhaus, B. L. Miller, et al.
Oculomotor function in frontotemporal lobar degeneration, related disorders and Alzheimer's disease
Brain, May 1, 2008; 131(5): 1268 - 1281.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
J. van der Zee, H. Urwin, S. Engelborghs, M. Bruyland, R. Vandenberghe, B. Dermaut, T. De Pooter, K. Peeters, P. Santens, P. P. De Deyn, et al.
CHMP2B C-truncating mutations in frontotemporal lobar degeneration are associated with an aberrant endosomal phenotype in vitro
Hum. Mol. Genet., January 15, 2008; 17(2): 313 - 322.
[Abstract] [Full Text] [PDF]

Home page
 Arch NeurolHome page
V. A. Cardenas, A. L. Boxer, L. L. Chao, M. L. Gorno-Tempini, B. L. Miller, M. W. Weiner, and C. Studholme
Deformation-Based Morphometry Reveals Brain Atrophy in Frontotemporal Dementia
Arch Neurol, June 1, 2007; 64(6): 873 - 877.
[Abstract] [Full Text] [PDF]

Home page
 BrainHome page
K. A. Josephs, J. R. Duffy, E. A. Strand, J. L. Whitwell, K. F. Layton, J. E. Parisi, M. F. Hauser, R. J. Witte, B. F. Boeve, D. S. Knopman, et al.
Clinicopathological and imaging correlates of progressive aphasia and apraxia of speech
Brain, June 1, 2006; 129(6): 1385 - 1398.
[Abstract] [Full Text] [PDF]

Home page
 BrainHome page
T. H. Bak, D. Yancopoulou, P. J. Nestor, J. H. Xuereb, M. G. Spillantini, F. Pulvermuller, and J. R. Hodges
Clinical, imaging and pathological correlates of a hereditary deficit in verb and action processing
Brain, February 1, 2006; 129(2): 321 - 332.
[Abstract] [Full Text] [PDF]

Home page
 BrainHome page
A. Kertesz, P. McMonagle, M. Blair, W. Davidson, and D. G. Munoz
The evolution and pathology of frontotemporal dementia
Brain, September 1, 2005; 128(9): 1996 - 2005.
[Abstract] [Full Text] [PDF]


Disclaimer:
Please note that abstracts for content published before 1996 were created through digital scanning and may therefore not exactly replicate the text of the original print issues. All efforts have been made to ensure accuracy, but the Publisher will not be held responsible for any remaining inaccuracies. If you require any further clarification, please contact our Customer Services Department.