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From: TSS ()
Subject: Clinical course in young patients with sporadic Creutzfeldt-Jakob disease
Date: July 24, 2005 at 8:27 am PST

Original Article
Clinical course in young patients with sporadic Creutzfeldt-Jakob disease
Constanze Boesenberg 1, Walter J. Schulz-Schaeffer, MD 2, Bettina Meissner, MD 1, Kai Kallenberg, MD 3, Mario Bartl 1, Uta Heinemann 1, Anna Krasnianski, MD 1, Katharina Stoeck, MD 1, Daniela Varges 1, Otto Windl, MD 4, Hans A. Kretzschmar, MD, FRCPath 5, Inga Zerr, MD 1 *
1Neurologische Klinik und Poliklinik der Georg-August-Universität Göttingen, Göttingen, Germany
2Institut für Neuropathologie der Georg-August-Universität Göttingen, Göttingen, Germany
3Abteilung für Neuroradiologie der Georg-August-Universität Göttingen, Göttingen, Germany
4Veterinary Laboratories Agency-Weybridge, Woodham Lane, New Haw, Addlestone, United Kingdom
5Institut für Neuropathologie, LMU München, München, Germany

email: Inga Zerr (

*Correspondence to Inga Zerr, Georg-August-Universität, Neurologische Klinik und Poliklinik, Robert-Koch-Strasse 40, 37075 Göttingen, Germany

Funded by:
Bundesministerium für Gesundheit und Soziales (BMGS); Grant Number: Az325-4471-02/15
Bundesministerium für Bildung und Forschung (BMBF); Grant Number: D1GI0301
European Commission; Grant Number: QLG3-CT-2002-81606

Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare neurodegenerative disease with the greatest incidence occurring in patients between 60 and 70 years old. Younger patients may also be affected. In this study, we used all case material available from 52 patients with sCJD aged 50 years or younger at disease onset, who were identified between 1993 and 2003 in Germany. The objective of this study was to describe the psychiatric and neurological features of these young patients with emphasis on the different codon 129 genotypes and PrP types, and to compare them with elder patients with sCJD and patients with variant CJD. We also gave particular attention to electroencephalogram, magnetic resonance imaging, and 14-3-3 results, as well as to the neuropathological lesion profile. The clinical syndrome in young patients differs from elder patients with CJD with respect to clinical signs, disease duration, technical investigations, and neuropathological lesion profile. The psychiatric symptoms in young patients with sCJD are similar to the psychiatric symptoms expressed by patients with variant CJD; however, in contrast with the variant cases, young patients with sCJD experience development of prominent dementia early in the disease course. Ann Neurol 2005

Received: 19 October 2004; Revised: 1 June 2005; Accepted: 1 June 2005
Digital Object Identifier (DOI)

10.1002/ana.20568 About DOI


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