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

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_BACK_PAGE,F2400_P1001_ARCHIVE_NUMBER,F2400_P1001_USE_ARCHIVE:1202,20081103.3458,Y



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-associated.html



http://www.wellsphere.com/cjd-article/medical-procedures-and-risk-for-sporadic-creutzfeldt-jakob-disease-japan-1999-2008-warning-to-neurosurgeons-and-ophthalmologi/641229



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-risk-for.html



Tuesday, August 11, 2009

Characteristics of Established and Proposed Sporadic Creutzfeldt-Jakob Disease Variants

http://creutzfeldt-jakob-disease.blogspot.com/2009/08/characteristics-of-established-and.html



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

Update: Creutzfeldt-Jakob Disease Associated with Cadaveric Dura Mater Grafts – Japan, 1979-2008 PRESS CONTACT: CDC Division of Media Relations (404) 639-3286

Because of the long incubation period, possibly exceeding 24.8 years, between the receipt of a dural graft (a cadaver-derived product used by neurosurgeons to patch defects in the substance covering a patient's brain) and symptom onset, Creutzfeldt-Jakob disease cases associated with dural grafts continue to be identified in Japan, despite preventive measures taken decades earlier. This MMWR submission updates the current status of an ongoing outbreak of dural graft-associated Creutzfeldt-Jakob disease in Japan. CDC played a key role in eliminating the source of the worldwide outbreak in 1987 when the first US case was reported. Despite the manufacturer of the implicated product revising its collection and production procedures at that time as a result of CDC's investigation, cases continue to be identified due to a long incubation period that may exceed 24.8 years. To date, 132 cases have been reported in Japan, with exposures having occurred during the period 1978-1993. In contrast, only 4 dural graft-associated Creutzfeldt-Jakob disease cases have been reported in the United States.

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U.S. DEPARTMENT OF HEALTH AND HUMAN SERVICES



http://www.cdc.gov/media/mmwrnews/2008/n081023.htm#3



Sunday, December 16, 2007

Risk factors for sporadic Creutzfeldt-Jakob disease

snip...

Dura mater grafts

The transmission of CJD from patient to patient by cadaveric dura mater grafts was first recognised in 1987 and there have now been at least 136 cases world-wide, including 88 cases identified in Japan7. Almost all of these cases have involved the insertion of Lyodura grafts produced by B. Braun Melsungen AG and processed before May 1987. Exceptions to the use of Lyodura grafts include one case in which the source was unknown, one case in which locally produced dura was implanted and one case associated with Tutoplast dura. The risk of transmission of CJD through dura mater grafts had been thought to be low because only a small number of recipients would receive contaminated material from any individual infected donor. The large number of Lyodura-associated cases of CJD suggests that there may have been cross-contamination during the production process.

For dura mater-associated cases of CJD in Japan, the mean latency period from receipt of the graft to the onset of CJD was 8.2 years (range, 1.3–16.1 years)8 and for the 114 reported cases world-wide by the millennium, the interval from the implantation of the graft to the development of clinical disease ranged from 1.5–18 years with a mean of about 6 years9. The risk of developing CJD after exposure to a dura mater graft is difficult to estimate because of limited information on the number of recipients. The major risk, however, appears to be in those individuals who received grafts between 1981 and 1987 (Fig. 1) and in Japan the minimum risk has been estimated at approximately one case of CJD per 3000 Lyodura graft recipients.

The majority of patients with dura mater-related CJD present with symptoms and signs consistent with sporadic CJD, but in some cases the presentation is less typical. A cerebellar syndrome has been described occasionally, but this does not necessarily correlate with the anatomical site of the original graft.

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http://creutzfeldt-jakob-disease.blogspot.com/2007/12/risk-factors-for-sporadic-creutzfeldt.html



ONE HUNDRED AND FIRST MEETING OF THE SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE

Oct 23, 2008 at 9:00 AM



http://seac992007.blogspot.com/2008/10/one-hundred-and-first-meeting-of_23.html



http://flounder068.vox.com/library/post/one-hundred-and-first-meeting-of-the-spongiform-encephalopathy-advisory-committee.html



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