Clinical course in young patients with sporadic Creutzfeldt-Jakob disease.
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Clinical course in young patients with sporadic Creutzfeldt-Jakob disease. / Boesenberg, Constanze; Schulz-Schaeffer, Walter J; Meissner, Bettina; Kallenberg, Kai; Bartl, Mario; Heinemann, Uta; Krasnianski, Anna; Stoeck, Katharina; Varges, Daniela; Windl, Otto; Kretzschmar, Hans A; Zerr, Inga.
in: ANN NEUROL, Jahrgang 58, Nr. 4, 4, 2005, S. 533-543.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - Clinical course in young patients with sporadic Creutzfeldt-Jakob disease.
AU - Boesenberg, Constanze
AU - Schulz-Schaeffer, Walter J
AU - Meissner, Bettina
AU - Kallenberg, Kai
AU - Bartl, Mario
AU - Heinemann, Uta
AU - Krasnianski, Anna
AU - Stoeck, Katharina
AU - Varges, Daniela
AU - Windl, Otto
AU - Kretzschmar, Hans A
AU - Zerr, Inga
PY - 2005
Y1 - 2005
N2 - 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.
AB - 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.
M3 - SCORING: Zeitschriftenaufsatz
VL - 58
SP - 533
EP - 543
JO - ANN NEUROL
JF - ANN NEUROL
SN - 0364-5134
IS - 4
M1 - 4
ER -
