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Unique clinicopathological features and PrP profiles in the first autopsied case of dura mater graft-associated Creutzfeldt–Jako

Posted Aug 12 2009 10:55pm
Articles in Press

ABSTRACT

Unique clinicopathological features and PrP profiles in the first autopsied case of dura mater graft-associated Creutzfeldt–Jakob disease with codon 219 lysine allele observed in Japanese population

Masamichi Ikawaa, Makoto Yonedaa, Akiko Matsunagaa, Hiroto Nakagawaa, Asuka Kazama-Suzukib, Nobuo Miyashitab, Hironobu Naikic, Tetsuyuki Kitamotod, Masaru Kuriyamaa

Received 2 April 2009; received in revised form 22 June 2009; accepted 23 July 2009. published online 10 August 2009. Corrected Proof

Abstract Polymorphism at codon 219 lysine in prion protein (PrP) is considered to affect the clinicopathological features of prion diseases including Creutzfeldt–Jakob disease (CJD) and to have an inhibiting effect on the pathogenesis of these diseases. We describe the first autopsied case of dura mater graft-associated CJD (dCJD) with heterozygosity of lysine at codon 219 in PrP observed in a Japanese subject. Although this case demonstrated the non-plaque type of dCJD and MM1 subgroup of CJD pathologically and biochemically, the patient demonstrated a long incubation period (19.3years), atypical periodic sharp-wave complexes with a dominant rhythm on EEG, partially scattered small deposits of plaque-like PrP along with synaptic type deposits of PrP on immunohistochemistry and an atypical MM1 glycosylation pattern with a relatively increased diglycosylated isoform of proteinase-resistant PrP on western blot analysis (i.e. “MM1 variant” pattern). These findings in this case were atypical of the non-plaque type of dCJD and MM1 subgroup of CJD. Thus, these findings can be unique to dCJD with codon 219 lysine allele, and this allele may influence the clinicopathological features and PrP profiles in dCJD.

Keywords: Dura mater graft-associated Creutzfeldt–Jakob disease, Codon 219 polymorphism, Glycosylation pattern in prion protein a Second Department of Internal Medicine (Neurology), Faculty of Medical Sciences, University of Fukui, 23-3 Shimoaiduki, Matsuoka, Eiheiji-cho, Yoshida-gun, Fukui 910-1193, Japan

b Department of Neurology, Koshigaya Municipal Hospital, 10-47-1 Higashi-Koshigaya, Koshigaya, Saitama 343-8577, Japan

c Department of Molecular Pathology, Faculty of Medical Sciences, University of Fukui, 23-3 Shimoaiduki, Matsuoka, Eiheiji-cho, Yoshida-gun, Fukui 910-1193, Japan

d Division of CJD Science and Technology, Department of Prion Research, Center for Translational and Advanced Animal Research on Human Diseases, Tohoku University Graduate School of Medicine, 2-1 Seiryo-cho, Aoba-ku, Sendai, Miyagi 980-8575, Japan

Corresponding author. Tel.: +81 776 61 8351; fax: +81 776 61 8110.

PII: S0022-510X(09)00741-2

doi:10.1016/j.jns.2009.07.019

© 2009 Elsevier B.V. All rights reserved

http://www.jns-journal.com/article/S0022-510X(09)00741-2/abstract



PLEASE NOTE ;

the patient demonstrated a long incubation period (19.3years)

[7] CJD in dura mater grafts - Japan, 1978-2008 Date Fri 24 Oct 2008 Source: MMWR Weekly 57(42);1152-1154 [edited]

http://www.cdc.gov/mmwr/preview/mmwrhtml/mm5742a3.htm



http://www.promedmail.org/pls/otn/f?p=2400:1001:2012455953418954::::F2400_P1001_



Thursday, October 23, 2008 Creutzfeldt-Jakob Disease Associated with Cadaveric Dura Mater Grafts - Japan, 1979-2008 : UPDATE

http://creutzfeldt-jakob-disease.blogspot.com/2008/10/creutzfeldt-jakob-disease-



http://www.wellsphere.com/cjd-article/medical-procedures-and-risk-for-sporadic-c



Thursday, January 29, 2009

Medical Procedures and Risk for Sporadic Creutzfeldt-Jakob Disease, Japan, 1999-2008 (WARNING TO Neurosurgeons and Ophthalmologists) Volume 15, Number 2-February 2009 Research

http://creutzfeldt-jakob-disease.blogspot.com/2009/01/medical-procedures-and-ris



Tuesday, August 11, 2009

Characteristics of Established and Proposed Sporadic Creutzfeldt-Jakob Disease Variants

http://creutzfeldt-jakob-disease.blogspot.com/2009/08/characteristics-of-establi



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