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MRI and clinical syndrome in dura materrelated Creutzfeldt-Jakob disease


Meissner, B; Kallenberg, K; Sanchez-Juan, P; Ramljak, S; Krasnianski, A; Heinemann, U; Eigenbrod, S; Gelpi, E; Barsic, B; Kretzschmar, H A; Schulz-Schaeffer, W J; Knauth, M; Zerr, I (2009). MRI and clinical syndrome in dura materrelated Creutzfeldt-Jakob disease. Journal of Neurology, 256(3):355-363.

Abstract

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.

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.

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Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > Institute for Regenerative Medicine (IREM)
Dewey Decimal Classification:610 Medicine & health
Date:2009
Deposited On:17 Jul 2009 13:33
Last Modified:16 Aug 2016 10:13
Publisher:Springer
ISSN:0340-5354
Additional Information:The original publication is available at www.springerlink.com
Publisher DOI:10.1007/s00415-009-0026-z
PubMed ID:19159063
Permanent URL: http://doi.org/10.5167/uzh-19661

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