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From: TSS ()
Subject: Sporadic Creutzfeldt-Jakob Disease Getting National Attention
Date: February 21, 2005 at 7:42 am PST

-------- Original Message --------
Subject: Sporadic Creutzfeldt-Jakob Disease Getting National Attention
Date: Mon, 21 Feb 2005 08:49:10 -0600
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy

##################### Bovine Spongiform Encephalopathy #####################

News Released: February 21, 2005

Sporadic Creutzfeldt-Jakob Disease Getting National Attention

(PRLEAP.COM) CJD Aware! is a North Carolina-based, information
organization that was started in the spring of 2001 after the death of
the founders mother from the sporadic form of Creutzfeldt-Jakob disease
on November 12, 2000. "The response we received last year was
wonderful," said Christy Brom, founder of CJD Aware! "In addition to a
CJD proclamation from the Governor of the state of North Carolina,
Michael F. Easley, we received proclamations
from (22) states including: Arkansas, Alabama, Colorado, Hawaii,
Illinois, Indiana, Kansas, Louisiana, Michigan, Missouri, Nebraska, New
Hampshire, New Jersey, Tennessee, Washington State and Wisconsin. A
formal letter of recognition from the Governor of California, Arnold
Schwarzenegger, was received on behalf of the state of California
applauding our efforts towards this cause. National CJD Awareness Week
will be an annual event for CJD Aware!"

"International CJD Day" was inaugurated on November 12, 2002 by The CJD
Support Network, a Great Britain-based support group founded in 1994 by
relatives of people with Creutzfeldt-Jakob disease. At that time,
Gillian Turner, Nationa CJD Case Coordinator for the Support
Network, invited CJD Aware!, and other international CJD support groups,
to join them in raising awareness about this disease annually on
November 12 in their communities.

Although Creutzfeldt-Jakob disease is a rare, neurological disorder,
great strides are currently being made to change that through
comprehensive clinical, educational and research environments. CJD is
often misdiagnosed as Alzheimers disease and is an infectious,
horrendous, brain-
deteriorating disease for which there is no treatment or cure.

CJD Aware! has a CJD information packet with informational
brochures/newsletters that are simple and easy to read. They go out to
hospitals, healthcare professionals, and to individuals who
contact the organization wishing information about Creutzfeldt-Jakob
disease. The packet includes a Resource brochure that lists other
organizations/websites where people can go and get further qualified
medical information that will hopefully assist them in understanding this
disease better.

Contact Information

Christy Brom
CJD Aware!
Email CJD Aware!


-------- Original Message -------- Subject: re-BSE prions propagate as

either variant CJD-like or sporadic CJD Date: Thu, 28 Nov 2002 10:23:43

-0000 From: "Asante, Emmanuel A" To:

Dear Terry,

I have been asked by Professor Collinge to respond to your request. I am

a Senior Scientist in the MRC Prion Unit and the lead author on the

paper. I have attached a pdf copy of the paper for your attention. Thank

you for your interest in the paper.

In respect of your first question, the simple answer is, yes. As you

will find in the paper, we have managed to associate the alternate

phenotype to type 2 PrPSc, the commonest sporadic CJD.

It is too early to be able to claim any further sub-classification in

respect of Heidenhain variant CJD or Vicky Rimmer's version. It will

take further studies, which are on-going, to establish if there are

sub-types to our initial finding which we are now reporting. The main

point of the paper is that, as well as leading to the expected new

variant CJD phenotype, BSE transmission to the 129-methionine genotype

can lead to an alternate phenotype which is indistinguishable from type

2 PrPSc.

I hope reading the paper will enlighten you more on the subject. If I

can be of any further assistance please to not hesitate to ask. Best wishes.

Emmanuel Asante

<> ____________________________________

Dr. Emmanuel A Asante MRC Prion Unit & Neurogenetics Dept. Imperial

College School of Medicine (St. Mary's) Norfolk Place, LONDON W2 1PG

Tel: +44 (0)20 7594 3794 Fax: +44 (0)20 7706 3272 email: (until 9/12/02)

New e-mail: (active from now)



full text ;

THE new findings of BASE in cattle in Italy of Identification of a
second bovine amyloidotic spongiform encephalopathy: Molecular
similarities with sporadic Creutzfeldt-Jakob disease

Adaptation of the bovine spongiform encephalopathy agent to primates
and comparison with Creutzfeldt- Jakob disease: Implications for
human health

THE findings from Corinne Ida Lasmézas*, [dagger] , Jean-Guy Fournier*,
Virginie Nouvel*,

Hermann Boe*, Domíníque Marcé*, François Lamoury*, Nicolas Kopp [Dagger

] , Jean-Jacques Hauw§, James Ironside¶, Moira Bruce [||] , Dominique

Dormont*, and Jean-Philippe Deslys* et al, that The agent responsible
for French iatrogenic growth hormone-linked CJD taken as a control is
very different from vCJD but is similar to that found in one case of
sporadic CJD and one sheep scrapie isolate;

Characterization of two distinct prion strains
derived from bovine spongiform encephalopathy
transmissions to inbred mice

Tissue distribution of protease resistant prion protein in variant

Creutzfeldt-Jakob disease using a highly sensitive immunoblotting assay.

Wadsworth JD, Joiner S, Hill AF, Campbell TA, Desbruslais M, Luthert

PJ, Collinge J.

MRC Prion Unit and Department of Neurogenetics, Imperial College

School of Medicine at St Mary's, Norfolk Place, W2 1PG, London, UK.

BACKGROUND: Variant Creutzfeldt-Jakob disease (vCJD) has a

pathogenesis distinct from other forms of human prion disease:

disease-related prion protein (PrP(Sc)) is readily detectable in

lymphoreticular tissues. Quantitation of risk of secondary

transmission, and targeting of risk reduction strategies, is limited

by lack of knowledge about relative prion titres in these and other

peripheral tissues, the unknown prevalence of preclinical vCJD, and

a transmission barrier which limits the sensitivity of bioassay. We

aimed to improve immunoblotting methods for high sensitivity

detection of PrP(Sc) to investigate the distribution of PrP(Sc) in a

range of vCJD tissues. METHODS: We obtained tissues at necropsy from

four patients with neuropathologically confirmed vCJD and from

individuals without neurological disease. Tissues were analysed by

sodium phosphotungstic acid precipitation of PrP(Sc) and western

blotting using high sensitivity enhanced chemiluminescence.

FINDINGS: We could reliably detect PrP(Sc) in the equivalent of 50

nL 10% vCJD brain homogenate, with a maximum limit of detection

equivalent to 5 nl. PrP(Sc) could be detected in tissue homogenates

when present at concentrations 10(4)-10(5) fold lower than those

reported in brain. Tonsil, spleen, and lymph node were uniformly

positive for PrP(Sc) at concentrations in the range of 0.1-15% of

those found in brain: the highest concentrations were consistently

seen in tonsil. PrP(Sc) was readily detected in the retina and

proximal optic nerve of vCJD eye at levels of 2.5 and 25%,

respectively of those found in brain. Other peripheral tissues

studied were negative for PrP(Sc) with the exception of low

concentrations in rectum, adrenal gland, and thymus from a single

patient with vCJD. vCJD appendix and blood (Buffy coat fraction)

were negative for PrP(Sc) at this level of assay sensitivity.

INTERPRETATION: We have developed a highly sensitive immunoblot

method for detection of PrP(Sc) in vCJD tissues that can be used to

provide an upper limit on PrP(Sc) concentrations in peripheral

tissues, including blood, to inform risk assessment models. Rectal

and other gastrointestinal tissues should be further investigated to

assess risk of iatrogenic transmission via biopsy instruments.

Ophthalmic surgical instruments used in procedures involving optic

nerve and the posterior segment of the eye, in particular the

retina, might represent a potential risk for iatrogenic transmission

of vCJD. Tonsil is the tissue of choice for diagnostic biopsy and

for population screening of surgical tissues to assess prevalence of

preclinical vCJD infection within the UK and other populations.

PMID: 11476832 [PubMed - indexed for MEDLINE]

Creutzfeldt-Jakob disease and inclusion body myositis: Abundant
disease-associated prion protein in muscle

Gabor G. Kovacs, MD PhD 1 2, Elisabeth Lindeck-Pozza, MD 1, Leila
Chimelli, MD, PhD 3, Abelardo Q. C. Araújo, MD, PhD 4, Alberto A.
Gabbai, MD, PhD 5, Thomas Ströbel, PhD 1, Markus Glatzel, MD 6, Adriano
Aguzzi, MD, PhD 6, Herbert Budka, MD 1 *

1Institute of Neurology, University of Vienna, and Austrian Reference
Centre for Human Prion Diseases, Vienna, Austria

2National Institute of Psychiatry and Neurology, Budapest, Hungary

3Department of Pathology, School of Medicine, Federal University of Rio
de Janeiro

4Department of Neurology, School of Medicine, Federal University of Rio
de Janeiro

5Department of Neurology, School of Medicine, Federal University of Sao
Paulo, Brazil

6Institute of Neuropathology, University Hospital of Zürich, Zürich,

email: Herbert Budka (

*Correspondence to Herbert Budka, Institute of Neurology, AKH 4J,
Wühringer Gürtel 18-20, POB 48, A-1097 Vienna, Austria

Funded by:

European Union (EU) Project; Grant Number: TSELAB QLK2-CT-2002-81523

EU Concerted Action PRIONET; Grant Number: QLK2-2000-CT-00837


Pathologicalprion protein (PrPSc) is the hallmark of prion diseases
affecting primarily the central nervous system. Using
immunohistochemistry, paraffin-embedded tissue blot, and Western blot,
we demonstrated abundant PrPSc in the muscle of a patient with sporadic
Creutzfeldt-Jakob disease and inclusion body myositis. Extraneural
PrPC-PrPSc conversion in Creutzfeldt-Jakob disease appears to become
prominent when PrPC is abundantly available as substrate, as in
inclusion body myositis muscle.


Received: 16 June 2003; Revised: 11 September 2003; Accepted: 11
September 2003

Digital Object Identifier (DOI)

10.1002/ana.10813 About DOI

NINDS Inclusion Body Myositis Information Page

AS Professor Aguzzi kindly put it most recently ;


Vet Pathol 42:107 108 (2005)

Letters to the Editor


Absence of evidence is not always evidence of absence.

In the article Failure to detect prion protein (PrPres) by

immunohistochemistry in striated muscle tissues of animals

experimentally inoculated with agents of transmissible spongiform

encephalopathy, recently published in Veterinary

Pathology (41:78 81, 2004), PrPres was not detected in striated

muscle of experimentally infected elk, cattle, sheep, and

raccoons by immunohistochemistry (IHC). Negative IHC,

however, does not exclude the presence of PrPSc. For example,

PrPres was detected in skeletal muscle in 8 of 32

humans with the prion disease, sporadic Creutzfeldt-Jakob

disease (CJD), using sodium phosphotungstic acid (NaPTA)

precipitation and western blot.1 The NaPTA precipitation,

described by Wadsworth et al.,3 concentrates the abnormal

isoform of the prion, PrPres, from a large tissue homogenate

volume before western blotting. This technique has increased

the sensitivity of the western blot up to three orders

of magnitude and could be included in assays to detect

PrPres. Extremely conspicuous deposits of PrPres in muscle

were detected by IHC in a recent case report of an individual

with inclusion body myositis and CJD.2 Here, PrPres was

detected in the muscle by immunoblotting, IHC, and paraf-

fin-embedded tissue blot. We would therefore caution that,

in addition to IHC, highly sensitive biochemical assays and

bioassays of muscle are needed to assess the presence or

absence of prions from muscle in experimental and natural

TSE cases.

Christina Sigurdson, Markus Glatzel, and Adriano Aguzzi

Institute of Neuropathology

University Hospital of Zurich

Zurich, Switzerland


1 Glatzel M, Abela E, et al: Extraneural pathologic prion

protein in sporadic Creutzfeldt-Jakob disease. N Engl J

Med 349(19):1812 1820, 2003

2 Kovacs GG, Lindeck-Pozza E, et al: Creutzfeldt-Jakob

disease and inclusion body myositis: abundant diseaseassociated

prion protein in muscle. Ann Neurol 55(1):

121 125, 2004

3 Wadsworth JDF, Joiner S, et al: Tissue distribution of protease

resistant prion protein in variant CJD using a highly

sensitive immuno-blotting assay. Lancet 358:171 180,


ALL animals for human/animal consumption must be tested for TSE.

ALL human TSEs must be made reportable Nationally and Internationally...


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