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This Article Figures Only Full Text Full Text (PDF) CME: Take the course for this article: Volume 65, Number 10, November 22, 2005 Data Supplement All Versions of this Article: 01.wnl.0000184674.32924.c9v1 65/10/1544    most recent Correspondence: Submit a response Alert me when this article is cited Alert me when Correspondence are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Meissner, B. Articles by Zerr, I. Search for Related Content PubMed PubMed Citation Articles by Meissner, B. Articles by Zerr, I.

Sporadic Creutzfeldt–Jakob disease

Clinical and diagnostic characteristics of the rare VV1 type

From the Departments of Neurology (Drs. Meissner, Krasnianski, Bartl, Zerr, D. Varges and C. Bösenberg), Neuroradiology (Drs. Kallenberg and Knauth), and Neuropathology (Dr. Schulz-Schaeffer), Georg-August-Universität Göttingen; and Center for Neuropathology and Prion Research (Drs. Westner and Kretzschmar), Ludwig-Maximilians-Universität München, Germany.

Address correspondence and reprint requests to Dr. Inga Zerr, Department of Neurology, University of Göttingen, Robert-Koch-Str. 40, D-37073 Göttingen, Germany; e-mail: IngaZerr{at}med.uni-goettingen.de

Background: Recently, six molecular subtypes of sporadic CJD (sCJD) have been identified showing differences regarding the disease course, neuropathologic lesion patterns, and sensitivity to diagnostic tools. Only isolated cases of the rare VV1 type have been reported so far.

Objective: To describe the clinical characteristics and neuropathologic lesion profiles in nine cases.

Methods: In the years 1993 until late 2003, 571 definite neuropathologically confirmed cases of sporadic CJD were identified in Germany. Of these, nine were homozygous for valine and displayed type 1 of the pathologic PrP^Sc in the brain (VV1 type).

Results: The authors describe eight men and one woman belonging to the VV1 type. All patients were relatively young at disease onset (median 44 years vs 65 years in all sCJD) with prolonged disease duration (median 21 months vs 6 months in all sCJD). During the initial stages, their main clinical signs were personality changes and slowly progressive dementia as well as focal neurologic deficits. None of the nine VV1 patients had periodic sharp-wave complexes (PSWCs) in the EEG. Only two out of seven displayed the typical signal increase of the basal ganglia on MRI, whereas signal increase of the cortex was seen in all patients. The 14-3-3 protein levels were elevated in CSF in all cases tested.

Conclusions: The clinical diagnosis of the VV1 type of sCJD can be best supported by the 14-3-3 test and cortical signal increase on MRI. Because of the young age at onset vCJD is sometimes suspected as a differential diagnosis. MRI plays an important role in differentiating these two disease types and should be performed early during the disease course.

Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the November 22 issue to find the title link for this article.

Editorial, see page 1520

This article was previously published in electronic format as an Expedited E-Pub on October 12, 2005, at www.neurology.org.

Supported by grants from the Bundesministerium für Bildung und Forschung (BMBF 01GI0301 and KZ: 0312720), the European Commission (EC) (QLG3-CT-2002-81606), and by the Bundesministerium für Gesundheit (BMG Az325-4471-02/15).

Disclosure: The authors report no conflicts of interest.

Received February 24, 2005. Accepted in final form August 24, 2005.

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