| A patient with MV2 subtype of sporadic Creutzfeldt-Jakob disease and atypical clinical presentation. | |
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MedLine Citation:
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PMID: 19130739 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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We report the case of a 71-year-old woman with progressive dementia over the course of 4 years, characterized by prominent pyramidal signs and by the lack of ataxia and other cerebellar signs. Creutzfeldt-Jakob disease (CJD) was not suspected during the patient's life. Autopsy brain tissue showed severe spongiform encephalopathy with kuru-like, but not florid, plaques in neocortex and cerebellum. Massive synaptic diffuse and plaque-like PrP(Sc) deposition was found in the cerebral cortex, striatum, cerebellum and brainstem. Genetic analysis revealed no PRNP gene mutations and methionine/valine heterozygosity (MV) at codon 129. The pathogenic scrapie prion protein (PrP(Sc)) pattern detected by Western blot was Type 2. However, this pattern showed a single unglycosylated band in contrast to the doublet described for MV2 subtype of sCJD with kuru plaques. In summary, this is an autopsy case report of a particular presentation of MV2 subtype of sCJD. |
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Authors:
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D Guerrero; N Martínez-Velilla; M C Caballero; M T Mendióroz; T Tuñón; J Masdeu; A Rodríguez; J Armstrong; I Ferrer |
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Publication Detail:
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Type: Case Reports; Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Clinical neuropathology Volume: 27 ISSN: 0722-5091 ISO Abbreviation: Clin. Neuropathol. Publication Date: 2008 Nov-Dec |
Date Detail:
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Created Date: 2009-01-09 Completed Date: 2009-02-10 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8214420 Medline TA: Clin Neuropathol Country: Germany |
Other Details:
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Languages: eng Pagination: 408-13 Citation Subset: IM |
Affiliation:
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Biomedical Research Center-Neurological Tissue Bank of Navarra, Irunlarrea, 3, Pamplona, 31008, Navarra, Spain. dguerres@cfnavarra.es |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Aged Creutzfeldt-Jakob Syndrome / diagnosis*, etiology*, metabolism Female Humans PrPSc Proteins / genetics, metabolism* |
| Chemical | |
Reg. No./Substance:
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0/PrPSc Proteins |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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