title: Clinical course in young patients with sporadic Creutzfeldt-Jakob disease.
abstract: 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.
authors.affiliation: Neurologische Klinik und Poliklinik der Georg-August-Universität Göttingen, Göttingen, Germany.
authors.forename: Constanze
authors.initials: C
authors.lastname: Boesenberg
authors.forename: Walter J
authors.initials: WJ
authors.lastname: Schulz-Schaeffer
authors.forename: Bettina
authors.initials: B
authors.lastname: Meissner
authors.forename: Kai
authors.initials: K
authors.lastname: Kallenberg
authors.forename: Mario
authors.initials: M
authors.lastname: Bartl
authors.forename: Uta
authors.initials: U
authors.lastname: Heinemann
authors.forename: Anna
authors.initials: A
authors.lastname: Krasnianski
authors.forename: Katharina
authors.initials: K
authors.lastname: Stoeck
authors.forename: Daniela
authors.initials: D
authors.lastname: Varges
authors.forename: Otto
authors.initials: O
authors.lastname: Windl
authors.forename: Hans A
authors.initials: HA
authors.lastname: Kretzschmar
authors.forename: Inga
authors.initials: I
authors.lastname: Zerr
mesh_terms: D048948:14-3-3 Proteins / Q000235:genetics / Q000378:metabolism*; D000328:Adult; D000367:Age Factors; D017668:Age of Onset; D000368:Aged; D015153:Blotting, Western / Q000379:methods; D001921:Brain / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology; D007562:Creutzfeldt-Jakob Syndrome / Q000453:epidemiology / Q000235:genetics / Q000378:metabolism* / Q000503:physiopathology*; D004569:Electroencephalography / Q000379:methods; D005260:Female; D005838:Genotype; D005858:Germany / Q000453:epidemiology; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D001523:Mental Disorders / Q000209:etiology / Q000503:physiopathology*; D008875:Middle Aged; D011328:Prions / Q000378:metabolism; D012189:Retrospective Studies; D012196:Review Literature as Topic
chemical_list: D048948:14-3-3 Proteins; D011328:Prions