Document Detail

MRI and clinical syndrome in dura mater-related Creutzfeldt-Jakob disease.
MedLine Citation:
PMID:  19159063     Owner:  NLM     Status:  MEDLINE    
OBJECTIVE: Iatrogenic Creutzfeldt-Jakob disease (iCJD) is mainly associated with dura mater (DM) grafts and administration of human growth hormones (hGH). Data on disease course in DM-CJD are limited. We describe the clinical and diagnostic findings in this patient group with special emphasis on MRI signal alterations. METHODS: Ten DM-CJD patients were studied for their clinical symptoms and diagnostic findings. The MRIs were evaluated for signal increase of the cortical and subcortical structures. RESULTS: DM-CJD patients had a median incubation time of 18 years and median disease duration of 7 months. The majority of patients were MM homozygous at codon 129 of the prion protein gene (PRNP) and presented with gait ataxia and psychiatric symptoms. No correlation between the graft site and the initial disease course was found. The MRI showed cortical and basal ganglia signal increase each in eight out of ten patients and thalamic hyperintensity in five out of ten cases. Of interest, patients with thalamic signal increase were homozygous for methionine. CONCLUSION: The MRI findings in DM-CJD largely resemble those seen in sporadic CJD, as the cortex and basal ganglia are mainly affected.
B Meissner; K Kallenberg; P Sanchez-Juan; S Ramljak; A Krasnianski; U Heinemann; S Eigenbrod; E Gelpi; B Barsic; H A Kretzschmar; W J Schulz-Schaeffer; M Knauth; I Zerr
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2009-01-23
Journal Detail:
Title:  Journal of neurology     Volume:  256     ISSN:  1432-1459     ISO Abbreviation:  J. Neurol.     Publication Date:  2009 Mar 
Date Detail:
Created Date:  2009-05-04     Completed Date:  2009-08-10     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0423161     Medline TA:  J Neurol     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  355-63     Citation Subset:  IM    
National TSE Reference Center, Dept. of Neurology, University of Göttingen, Robert-Koch-Str. 40, 37075, Göttingen, Germany.
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MeSH Terms
Ataxia / complications
Brain / pathology*
Brain Tissue Transplantation / adverse effects*
Creutzfeldt-Jakob Syndrome / complications*,  diagnosis,  pathology*
DNA Mutational Analysis
Dura Mater / transplantation*
Iatrogenic Disease*
Infectious Disease Incubation Period
Magnetic Resonance Imaging
Mental Disorders / complications
Middle Aged
Prions / genetics
Time Factors
Reg. No./Substance:
0/PRNP protein, human; 0/Prions

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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