Document Detail

Clinical course in young patients with sporadic Creutzfeldt-Jakob disease.
MedLine Citation:
PMID:  16037975     Owner:  NLM     Status:  MEDLINE    
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.
Constanze Boesenberg; Walter J Schulz-Schaeffer; Bettina Meissner; Kai Kallenberg; Mario Bartl; Uta Heinemann; Anna Krasnianski; Katharina Stoeck; Daniela Varges; Otto Windl; Hans A Kretzschmar; Inga Zerr
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Publication Detail:
Type:  Comparative Study; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Annals of neurology     Volume:  58     ISSN:  0364-5134     ISO Abbreviation:  Ann. Neurol.     Publication Date:  2005 Oct 
Date Detail:
Created Date:  2005-09-28     Completed Date:  2005-12-15     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  7707449     Medline TA:  Ann Neurol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  533-43     Citation Subset:  IM    
Neurologische Klinik und Poliklinik der Georg-August-Universität Göttingen, Göttingen, Germany.
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MeSH Terms
14-3-3 Proteins / genetics,  metabolism*
Age Factors
Age of Onset
Blotting, Western / methods
Brain / metabolism,  pathology,  physiopathology
Creutzfeldt-Jakob Syndrome / epidemiology,  genetics,  metabolism*,  physiopathology*
Electroencephalography / methods
Germany / epidemiology
Magnetic Resonance Imaging / methods
Mental Disorders / etiology,  physiopathology*
Middle Aged
Prions / metabolism
Retrospective Studies
Review Literature as Topic
Reg. No./Substance:
0/14-3-3 Proteins; 0/Prions

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