Skip Navigation



Brain Advance Access published online on May 23, 2006
Brain, doi:10.1093/brain/awl123
This Article
Right arrow FREE Full Text (PDF) Freely available
Right arrow All Versions of this Article:
129/9/2288    most recent
awl123v1
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 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 arrowRequest Permissions
Right arrow Disclaimer
Google Scholar
Right arrow Articles by Krasnianski, A.
Right arrow Articles by Zerr, I.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Krasnianski, A.
Right arrow Articles by Zerr, I.
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?

© The Author (2006). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org
Received November 25, 2005
Revised January 25, 2006
Accepted April 10, 2006

Article

Clinical findings and diagnostic tests in the MV2 subtype of sporadic CJD

Anna Krasnianski 1, Walter J. Schulz-Schaeffer 2, Kai Kallenberg 3, Bettina Meissner 1, Donald A. Collie 4, Sigrun Roeber 5, Mario Bartl 1, Uta Heinemann 1, Daniela Varges 1, Hans A. Kretzschmar 5, and Inga Zerr 1 *

1 Department of Neurology, Georg-August University Göttingen, Germany
2 Department of Neuropathology, Georg-August University Göttingen, Germany
3 Department of Neuroradiology, Georg-August University Göttingen, Germany
4 Department of Neuroradiology, Western General Hospital Edinburgh, UK
5 Department of Neuropathology, Ludwig-Maximillian University Munich, Germany

* To whom correspondence should be addressed.
Inga Zerr, E-mail: epicjd{at}med.uni-goettingen.de


  Abstract

Atypical clinical course and low sensitivity of established diagnostic tests are the main diagnostic problems in the MV2 subtype of sporadic Creutzfeldt-Jakob disease (sCJD). Clinical symptoms and signs, MRI, EEG and biochemical CSF markers were studied in 26 patients. Histological findings were semiquantitatively evaluated. Compared with typical sCJD, the disease duration was prolonged (median 12 months). Dementia, ataxia and psychiatric symptoms were present in all patients. Extrapyramidal signs were observed in 88%. T2-weighted MRI showed basal ganglia hyperintensities in 90%. Increased thalamic signal intensity was detected in 88% on diffusion-weighted MRI. Increased CSF tau-protein was found in 83%, and the 14-3-3 test was positive in 76%. The EEG revealed periodic sharp wave complexes in only two patients. Kuru plaques, severe thalamic and basal ganglia gliosis and spongiform changes, and neuronal loss in the pulvinar were the prominent histological features. At least one of the three diagnostic tests (MRI, tau- and 14-3-3 protein) supported the clinical diagnosis in all patients. MRI was the most sensitive of the diagnostic tests applied. Thalamic hyperintensities were observed unusually frequently. Prolonged disease duration, early and prominent psychiatric symptoms, absence of typical EEG, thalamic hyperintensities on MRI and relatively low 14-3-3 protein sensitivity may be suspicious for variant CJD. However, distinct sensory symptoms and young age at onset, which are often found in the latter, are not common in the MV2 subtype, and the pulvinar sign was observed in only one case.

Keywords: CJD; MV2 subtype; MRI; Pulvinar sign; diagnosis.
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
I. Cali, R. Castellani, A. Alshekhlee, Y. Cohen, J. Blevins, J. Yuan, J. P. M. Langeveld, P. Parchi, J. G. Safar, W.-Q. Zou, et al.
Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt-Jakob disease: its effect on the phenotype and prion-type characteristics
Brain, October 1, 2009; 132(10): 2643 - 2658.
[Abstract] [Full Text] [PDF]

Home page
 BrainHome page
R. Lodi, P. Parchi, C. Tonon, D. Manners, S. Capellari, R. Strammiello, R. Rinaldi, C. Testa, E. Malucelli, B. Mostacci, et al.
Magnetic resonance diagnostic markers in clinically sporadic prion disease: a combined brain magnetic resonance imaging and spectroscopy study
Brain, October 1, 2009; 132(10): 2669 - 2679.
[Abstract] [Full Text] [PDF]

Home page
 BrainHome page
I. Zerr, K. Kallenberg, D. M. Summers, C. Romero, A. Taratuto, U. Heinemann, M. Breithaupt, D. Varges, B. Meissner, A. Ladogana, et al.
Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease
Brain, October 1, 2009; 132(10): 2659 - 2668.
[Abstract] [Full Text] [PDF]

Home page
 NeurologyHome page
B. Meissner, K. Kallenberg, P. Sanchez-Juan, D. Collie, D. M. Summers, S. Almonti, S. J. Collins, P. Smith, P. Cras, G. H. Jansen, et al.
MRI lesion profiles in sporadic Creutzfeldt-Jakob disease
Neurology, June 9, 2009; 72(23): 1994 - 2001.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Neuroradiol.Home page
B. Meissner, K. Kallenberg, P. Sanchez-Juan, A. Krasnianski, U. Heinemann, D. Varges, M. Knauth, and I. Zerr
Isolated Cortical Signal Increase on MR Imaging as a Frequent Lesion Pattern in Sporadic Creutzfeldt-Jakob Disease
AJNR Am. J. Neuroradiol., September 1, 2008; 29(8): 1519 - 1524.
[Abstract] [Full Text] [PDF]

Home page
 BrainHome page
U. Heinemann, A. Krasnianski, B. Meissner, D. Varges, K. Kallenberg, W. J. Schulz-Schaeffer, B. J. Steinhoff, E. M. Grasbon-Frodl, H. A. Kretzschmar, and I. Zerr
Creutzfeldt-Jakob disease in Germany: a prospective 12-year surveillance
Brain, May 1, 2007; 130(5): 1350 - 1359.
[Abstract] [Full Text] [PDF]

Home page
 BrainHome page
M. W. Head and J. W. Ironside
Sporadic Creutzfeldt-Jakob disease: further twists and turns in a convoluted protein.
Brain, September 1, 2006; 129(Pt 9): 2238 - 2240.
[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.