Document Detail


A patient with MV2 subtype of sporadic Creutzfeldt-Jakob disease and atypical clinical presentation.
MedLine Citation:
PMID:  19130739     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
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:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Clinical neuropathology     Volume:  27     ISSN:  0722-5091     ISO Abbreviation:  Clin. Neuropathol.     Publication Date:    2008 Nov-Dec
Date Detail:
Created Date:  2009-01-09     Completed Date:  2009-02-10     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8214420     Medline TA:  Clin Neuropathol     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  408-13     Citation Subset:  IM    
Affiliation:
Biomedical Research Center-Neurological Tissue Bank of Navarra, Irunlarrea, 3, Pamplona, 31008, Navarra, Spain. dguerres@cfnavarra.es
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MeSH Terms
Descriptor/Qualifier:
Aged
Creutzfeldt-Jakob Syndrome / diagnosis*,  etiology*,  metabolism
Female
Humans
PrPSc Proteins / genetics,  metabolism*
Chemical
Reg. No./Substance:
0/PrPSc Proteins

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