Document Detail


Sporadic Creutzfeldt-Jakob disease: magnetic resonance imaging and clinical findings.
MedLine Citation:
PMID:  15314808     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVE: To assess if clinical features, prion protein codon 129, and molecular subtype correlate with MRI basal ganglia hyperintensity in sporadic Creutzfeldt-Jakob disease (CJD). METHODS: The authors studied 219 patients including 153 confirmed CJD cases for their neurologic symptoms and MRI findings. The MRI was assessed by a blinded investigator for the presence of high signal intensity on T2-weighted images in the basal ganglia. RESULTS: Patients with basal ganglia high signal on T2-weighted images were more likely to present with rapid progressive dementia in an early stage and shorter disease duration (median 6.7 months and 8.6 months). Surprisingly, among the CJD cases, patients without signal increase of the basal ganglia were shown to have a higher frequency of extrapyramidal disturbances (82% vs 70%). More striking differences were found for symptoms such as depression and sensory disturbances, which were more frequent among cases without signal increase. MRI was more likely to be diagnostic in patients with MV2 molecular subtype. CONCLUSIONS: Selected clinical and pathologic features correlate with the presence of basal ganglia high signal on T2-weighted MRI in patients with definite or probable CJD.
Authors:
B Meissner; K Körtner; M Bartl; U Jastrow; B Mollenhauer; A Schröter; M Finkenstaedt; O Windl; S Poser; H A Kretzschmar; I Zerr
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Publication Detail:
Type:  Comparative Study; Evaluation Studies; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Neurology     Volume:  63     ISSN:  1526-632X     ISO Abbreviation:  Neurology     Publication Date:  2004 Aug 
Date Detail:
Created Date:  2004-08-17     Completed Date:  2005-03-01     Revised Date:  2006-11-28    
Medline Journal Info:
Nlm Unique ID:  0401060     Medline TA:  Neurology     Country:  United States    
Other Details:
Languages:  eng     Pagination:  450-6     Citation Subset:  AIM; IM    
Affiliation:
Department of Neurology, University of Göttingen, Germany.
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MeSH Terms
Descriptor/Qualifier:
Adult
Aged
Amyloid / genetics
Basal Ganglia / pathology*
Brain Diseases / pathology
Codon / genetics
Creutzfeldt-Jakob Syndrome / classification,  diagnosis,  genetics,  mortality,  pathology*
DNA Mutational Analysis
Dementia / pathology
Diagnosis, Differential
Female
Genotype
Humans
Magnetic Resonance Imaging* / methods
Male
Middle Aged
PrPSc Proteins / genetics
Prions
Protein Precursors / genetics
Retrospective Studies
Single-Blind Method
Survival Analysis
Chemical
Reg. No./Substance:
0/Amyloid; 0/Codon; 0/PRNP protein, human; 0/PrPSc Proteins; 0/Prions; 0/Protein Precursors
Comments/Corrections
Comment In:
Neurology. 2005 Apr 12;64(7):1318; author reply 1318   [PMID:  15824383 ]
Neurology. 2004 Aug 10;63(3):410-1   [PMID:  15304568 ]

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