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From: TSS ()
Subject: Re: HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory
Date: March 13, 2006 at 2:36 pm PST

In Reply to: HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory posted by TSS on January 29, 2006 at 9:03 am:

WHO'S vCJD case definition should be revised
Mon Mar 13, 2006 16:56

Monday March 13 2006

The Lancet 2006; 367:874

The first Japanese case of variant Creutzfeldt-Jakob disease showing
periodic electroencephalogram

WHO'S vCJD case definition should be revised

Case Report

In February, 2004, a 50-year-old Japanese man was
referred to our Creutzfeldt-Jakob disease (CJD)
surveillance committee. In the first half of 1990, the
patient had spent about 24 days in the UK, 3 days in
France, and 2 weeks in other European countries where
variant CJD (vCJD) has not been reported. He had no
history of surgery or blood transfusion, or a family history
of prion disease. In June, 2001, aged 48 years, he had
difficulty in writing Chinese characters. In October, 2001,
he showed mental symptoms, such as irritability,
personality changes, and memory impairment, followed
by painful dysaesthesia in the legs, ataxia, dementia, and
abnormal behaviour. Retrospective review of an MRI
taken in August, 2002, showed slight hyperintensity in the
thalamus. In January, 2003, he showed dementia, ataxia,
and hyperreflexia. Brain MRI at that time showed
symmetrical hyperintensity of the thalamus. Electroencephalogram
(EEG) showed diffuse slowing, but no
periodic synchronous discharges (PSD). The cerebrospinal
fluid was positive for 14-3-3 protein. Analysis of the
prion protein (PrP) gene showed no mutation,
methionine/methionine at codon 129, and glutamic
acid/glutamic acid at codon 219. He showed rapid
deterioration of both motor and cognitive function.
In December, 2003, he developed akinetic mutism,
myoclonus, and pyramidal signs. Brain MRI showed
hyperintensity in the caudate, putamen, thalamus, and
cerebral cortex, with higher intensity in the caudate and
putamen than the thalamus. EEG suggested the presence
of PSD (figure, A). The diagnosis of probable sporadic
CJD was supported by EEG and MRI findings.1 He died of
pneumonia in December, 2004. Autopsy showed findings
characteristic of vCJD, including florid plaques (figure, B)
and the Parchi type 2B or Collinge type 4 pattern of
protease-resistant PrP (not shown).
This is the first Japanese case of definite vCJD. The
progressive neuropsychiatric disorder was consistent with
vCJD, although the illness duration was unusually long
compared with most vCJD cases reported to date (median
14 months).2 The findings 19 months after onset of
symptoms, showing the pulvinar sign on MRI and the
absence of PSD on EEG, together with the clinical
features, fulfilled the criteria of probable vCJD.3 However,
30 months after onset, PSD appeared on EEG, and the
pulvinar sign on MRI disappeared following an increase
in intensity of other grey matter nuclei, fulfilling the
criteria of probable sCJD.1 There have been no previous
reports of PSD on EEG in vCJD, although conversion of a
positive to negative pulvinar sign on MRI has been
described in a few vCJD patients.4 Our case shows that
PSD does not exclude the possibility of vCJD. We suggest
revision of the WHO vCJD case definition3 to prevent
missing cases of vCJD. It is unclear when our patient was
exposed to the infective agent. The BSE outbreak in the
UK was still increasing when he visited the UK, and it was
confirmed that he ate food containing mechanically
recovered meat that may be associated with contamination
with BSE agent from nervous tissue;5 however, exposure
in France, other European countries, and Japan cannot be
excluded. If he was exposed to the BSE agent in the UK
(exposure just once would be sufficient to cause vCJD), we
calculate the incubation period between such pinpoint
exposure and onset of vCJD to be 11·5 years.
Following the identification of this first Japanese case of vCJD by the CJD
Surveillance Committee, Japan, we constituted the vCJD Working Group
in the Committee. To protect the patient’s privacy, we have decided to
publish this report under the name of the vCJD Working Group, CJD
Surveillance Committee, Japan. The corresponding author is the chair of
both the vCJD Working Group and the CJD Surveillance Committee.
We thank the patient’s family for permission to publish this report. The
CJD Surveillance Committee is funded by the Ministry of Health, Labour
and Welfare, Japan; the funding source had no involvement in the process
of publication of this paper.
1 Global surveillance, diagnosis and therapy of human transmissible
spongiform encephalopathies: Report of a WHO consultation.
Geneva, Switzerland, 9–11 February 1998 (
resources/publications/bse/WHO_EMC_ZDI_98_9/en/) (accessed
Nov 30, 2005).
2 Will RG, Ward HJ. Clinical features of variant Creutzfeldt-Jakob
disease. Curr Top Microbiol Immunol 2004; 284: 121–32.
3 The revision of the surveillance case definition for variant
Creutzfeldt-Jakob Disease (vCJD). Report of a WHO consultation
Edinburgh, United Kingdom 17 May 2001 (
en/) (accessed Nov 30, 2005).
4 Collie DA, Summers DM, Sellar RJ, et al. Diagnosing variant
Creutzfeldt-Jakob disease with the pulvinar sign: MR imaging
findings in 86 neuropathlogically confirmed cases. Am J Neuroradiol
2003; 24: 1560–69.
5 Food Standards Agency. Agency study reports on historic use of
mechanically recovered meat in food 1980–1995 (2002) (http:// mrm)
(accessed Nov 30, 2005).
Lancet 2006; 367: 874
Creutzfeldt-Jakob Surveillance
Committee, Department of
Neurology and Neurobiology
of Aging, Kanazawa University
Graduate School of Medical
Science, 13-1, Takara-machi,
Kanazawa 920-8640, Japan
Correspondence to:
Prof Masahito Yamada
874 Vol 367 March 11, 2006
The first Japanese case of variant Creutzfeldt-Jakob disease
showing periodic electroencephalogram
Masahito Yamada on behalf of the Variant CJD Working Group,
Creutzfeldt-Jakob Disease Surveillance Committee, Japan
Figure: (A) EEG in August, 2004, 39 months after onset, showing PSD typical
of sporadic CJD. (B) The frontal
cortex showing florid plaques, severe spongiform changes, and neuronal loss
(HE, bar = 100 m).
PrP immunohistochemistry showed many PrP-positive plaques and PrP deposits
with a pericellular pattern
(not shown).
O2–A2 50 V
1 s


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