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From: TSS ()
Subject: BSE TESTING CREEKSTONE VS USDA BROWN & DETWILER DECLARATIONS
Date: November 11, 2006 at 3:03 pm PST

In Reply to: BSE TESTING CREEKSTONE VS USDA posted by TSS on November 11, 2006 at 3:00 pm:

Case 1:06-cv-00544-JR Document 14-9 Filed 11/03/2006 Page 1 of 47

Creekstone Farms Premium Beef v. USDA

Civ. Action No. 06-544 (JR)

Plaintiff’s Summary Judgment Reply and Opposition

EXHIBIT 8

Case 1:06-cv-00544-JR Document 14-9 Filed 11/03/2006 Page 2 of 47

IN THE UNITED STATES DISTRICT COURT

FOR THE DISTRICT OF COLUMBIA


)

CREEKSTONE FARMS PREMIUM BEEF, LLC, )

)

Plaintiff, )

)

vs. ) Civil Action No. 06-544 (JR)

)

UNITED STATES DEPARTMENT OF AGRICULTURE, )

and MIKE JOHANNS, IN HIS CAPACITY AS THE )

SECRETARY OF AGRICULTURE, )

)

Defendants. )

__________________________________________________)

DECLARATION OF PAUL W. BROWN, M.D.

Paul W. Brown, MD, certifies and states as follows:

1. My entire 43-year career has been devoted to the study of the
Transmissible

Spongiform Encephalopathies (TSEs, or ‘prion diseases’) in the National
Institutes of Health (NIH)

laboratory of Nobel Laureate D. Carleton Gajdusek; and for the last 15
years of my career I held the

position of Senior Investigator at the National Institute of
Neurological Disorders and Stroke

(NINDS) at the NIH. In this capacity I oversaw and conducted research
involving these invariably

fatal, neurodegenerative diseases, which include both bovine spongiform
encephalopathy (BSE),

commonly referred to as “mad cow” disease, and variant Creutzfeldt-Jakob
Disease (vCJD), a

human TSE infection acquired by the consumption of BSE-contaminated meat
products.

2. Since leaving the NIH two years ago, I have continued to engage in
research, writing,

and scientific advisory activities in various aspects of TSE. Ongoing
research projects include an

ultra-high pressure methodology to inactivate the agent of BSE; a
pre-clinical diagnostic blood

screening test for TSEs in general, and variant CJD in particular; and a
study to determine whether


Case 1:06-cv-00544-JR Document 14-9 Filed 11/03/2006 Page 3 of 47

blood infectivity in humans with CJD can be diluted to extinction
(important in the context of the

size of plasma pools used for the extraction of therapeutic proteins
such as anti-hemophilic factor). I

have continued to participate in scientific advisory boards, including
chairing the safety committee

of LFB (the plasma fractionator in France), co-chairing the scientific
research committee of a

pharmaceutical consortium to award research contracts, and advising the
EuroCJD committee on

global BSE and CJD surveillance, and the Argentine Agriculture
Department on BSE issues. I have

also continued to accept invitations to chair and/or lecture at numerous
scientific meetings on TSEs

in the Americas and Europe.

I am a member of the American College of Physicians, the American
Epidemiological

Society, the Infectious Diseases Society of America, the American
Society for Virology, the Société

Française de Neurologie, and the American Neurological Association. I
have authored or coauthored nearly 400 papers, mainly dealing with TSE,
and especially the topics of epidemiology,

infectivity, and inactivation. (A list of scientific papers (published
from 1991 to the present or that

are in press) of which I am an author, primarily regarding TSEs, is
attached as Attachment A.) Since

1990, I have been an Associate Editor of the European Journal of
Epidemiology, and from 1991 until

1997, was an Associate editor of the Journal of the Neurological Sciences.

3. Before entering the NIH in the US Public Health Service, I attended
Harvard College

(A.B., Magna cum Laude), and the Johns Hopkins School of Medicine (M.D.,
Alpha Omega Alpha

Honor Society), and undertook Medical Internship and Residency training
at both the Johns Hopkins

Hospital in Baltimore, Maryland, and the University of California School
of Medicine in San

Francisco, California. I am a Diplomate of the American Board of
Internal Medicine.

(My curriculum vitae is attached to this declaration as Attachment B.)

-2


Case 1:06-cv-00544-JR Document 14-9 Filed 11/03/2006 Page 4 of 47

4. At the request of William L. Miller of the William Miller Group, PLLC
(outside

counsel for Creekstone Farms Premium Beef, LLC), on October 11, 2004, I
agreed to prepare this

declaration regarding the current scientific understanding of bovine
spongiform encephalopathy

(BSE) in support of the litigation by Creekstone Farms Premium Beef, LLC
to be allowed to

voluntarily test for BSE in cattle processed at its plant in Arkansas
City, Kansas. Prior to October

11, I have had no relationship with Creekstone Farms. The opinions
expressed in this declaration are

based solely on my knowledge of BSE and other TSEs that I have gained
from my experience as a

physician and neuroscientist over the past 45 years.

5. Based on current science, we do not know with certainty how far in
advance of

displaying clinical signs of BSE a bovine animal could test positive
using a BSE rapid test such

as the rapid test technology developed by Bio-Rad Laboratories, Inc.,
and currently used by the

United States Department of Agriculture to test for BSE. For example,
the Canadian Food

Inspection Agency (CFIA) recently issued a report on a 50-month-old cow
from Alberta, Canada

that on July 13, 2006 was confirmed as BSE-infected. The CFIA reports
states as follows:

This animal was detected and diagnosed with BSE during a pre-clinical
phase of the

disease. The normal disease course to expression of clinical signs in
this animal would be

expected to have included an additional three to six months of
incubation followed by an

additional one to two months of clinical expression prior to being
recognized as

symptomatic of BSE and targeted for testing. Had an unrelated disease
not hastened her

entry into the surveillance stream, this animal would most likely have
demonstrated

clinical signs sometime between 54 and 56 months, not significantly
different from the

age range of previous cases.

-3


Case 1:06-cv-00544-JR Document 14-9 Filed 11/03/2006 Page 5 of 47

Canadian Food Inspection Agency, Report On The Investigation Of The
Seventh Case Of

Bovine Spongiform Encephalopathy (BSE) In Canada, available at

http://www.inspection.gc.ca/english/anima/heasan/disemala/bseesb/ab2006/7investe.shtml.


Thus, in this instance, had the cow not succumbed to an unrelated
disease and had a brain stem

sample submitted for BSE testing, it would probably have been four to
eight months longer

before the cow would have displayed symptoms that might have caused it
to be tested because of

suspicion of BSE.

6. Experimental studies to determine range of intervals between positive
tests and the

onset of clinical signs have not been done (a critical experiment has in
fact been done in the UK,

but only infectivity was measured, and tests for the presence of prion
protein have not been

done). Wells, G. A. H., S.A. Hawkins, R.B. Green, A.R. Austin, I.
Dexter, Y.I. Spencer, M.J.,

Chaplin, M.J. Stack, and M Dawson, 1998, Preliminary observations on the
pathogenesis of

experimental bovine spongiform encephalopathy (BSE): an update, Vet.
Rec. 142:103-106;

Wells, G.A.H., J. Spiropoulos, S.A.C. Hawkins, and S. J.Ryder, 2005,
Pathogenesis of

experimental bovine spongiform encephalopathy: preclinical infectivity
in tonsil and

observations on the distribution of lingual tonsil in slaughtered
cattle, Vet. Rec. 156:401-407.

Equally important, field experience in countries that have gone to
universal testing (including

universal testing above a certain age) has shown that such testing
(active surveillance) identifies

considerably more cases than only testing cattle whose behavior happens
to attract the attention

of authorities happens to attract the attention of authorities (passive
surveillance).

Report on the Monitoring and Testing of Ruminants for the Presence of
Transmissible

Spongiform Encephalopathy (TSE) in the EU in 2005, Final Annual Report,
2005, European

Commission, 20 June 2006. (Section 4.2, Chart B2), available at

-4


Case 1:06-cv-00544-JR Document 14-9 Filed 11/03/2006 Page 6 of 47

http://ec.europa.eu/food/food/biosafety/bse/annual_reps_en.htm.

7. Assumptions about the likely course of BSE infection in cattle and
its significance for

potential vCJD infection in humans who consume cattle tissues are
largely just that—

assumptions or extrapolations from limited data, not tested conclusions.
We have little or no

direct evidence of such things as when in life a bovine is most
susceptible to BSE; how much

less susceptible, if at all, humans are than cattle; whether shorter
incubation times in cattle are

the result of exposure to a high dose at a young age or some other
factor(s) (such as genetic

susceptibility or the strain of prions); how many humans may ultimately
be shown to have vCJD;

or how effective SRM removal is at reducing the risk of transmission of
BSE from an infected

animal.

8. The question of the interval between when cattle may test positive
for BSE and

when those cattle exhibit clinical signs of the disease has in any case
been made irrelevant by the

recent discovery of atypical cases of BSE that usually occur in older
asymptomatic animals that

for one reason or another are culled, and are then discovered to test
positive. Thus, some cattle

that on visual inspection appear normal are infected, and this fact
negates any argument about a

limited window of 'pre-clinical' positivity in BSE tests, as there are
no signs leading to a

suspicion of infection. The only way that these cattle are identified as
BSE-infected is through

testing. The frequency of such infections is not yet known, but so far
has ranged from less than

1% all the way up to 15% of the total number of test positives.
International Conference on

Prion Diseases of NeuroPrion, Network of Excellence, Prion2006, Turin,
Italy, 4-6 October

2006, Book of Abstracts (hereinafter “Prion2006 Abstracts”), See

http://www.neuroprion.com/en/ev_prion2006.html; Brown P, McShane LM,
Zanusso G,

Detwiler L, On the question of sporadic or atypical bovine spongiform
encephalopathy and

-5


Case 1:06-cv-00544-JR Document 14-9 Filed 11/03/2006 Page 7 of 47

Creutzfeldt-Jakob disease, Emerg. Inf. Dis., 2006, 12: 1816-21, in
press, December 2006. (A

copy of the article is attached to this declaration as Attachment C.)

9. Although these cases of asymptomatic, atypical BSE have been found in
older

cattle, we do not currently know the concentration and distribution of
infection and infectivity

that might have been found in these cattle at an earlier age. Another
issue concerns evidence that

atypical BSE may be more virulent for humans than typical BSE. This
evidence comes from

experimental transmission studies in at least 4 different laboratories.
All have shown more rapid

onset of disease (shorter incubation periods) following inoculation with
atypical BSE than with

typical BSE, and in one study, BSE did not transmit at all. These
studies involved the use of

wild-type mice, bovinized and humanized transgenic mice, and (most
worrisome) non-human

primates as recipient animals. Prion2006 Abstracts, See

http://www.neuroprion.com/en/ev_prion2006.html.

10. We do not have any direct evidence of how few BSE prions need to be
consumed

in order for a human to be at risk of vCJD. Just because the disease has
not progressed far

enough in a bovine to produce clinical symptoms does not mean that there
are insufficient

concentrations of BSE prions in that bovine's tissue to present a risk
of vCJD if consumed. (In

fact, logic suggests the contrary, because it would imply that all of
the people with vCJD were

infected by the consumption of meat products from cattle that were
displaying signs of BSE at

the time of slaughter, which seems highly unlikely.) The available tests
for BSE are generally

believed to have lower limits of detecting concentrations of prions
that, if ingested, could cause

BSE in other cattle, or potentially vCJD in humans. In cattle, the
minimum amount of tissue

needed to infect a cow by the oral route is only 0.001 gram (Dr. Gerald
Wells, personal

-6


Case 1:06-cv-00544-JR Document 14-9 Filed 11/03/2006 Page 8 of 47

communication), and this figure has been used for risk analyses in
humans (a hypothesized

bovine to human 'species barrier' effect has never been documented).

11. Just because cattle are under 30 months of age does not mean that
they present no

risk of BSE, both because of the facts discussed above about the
uncertain relationship between

testing positive for BSE and having sufficiently concentrated prions to
cause infection in

humans, and because BSE has been detected in younger cattle in Europe
and Japan.

Additionally, the way that the age of cattle to be slaughtered is often
determined (dentition) is

not very precise. As a matter of protecting animal and human health from
the fatal consequences

as BSE, detecting even a handful of cases that otherwise would be missed
is very valuable.

USDA requires testing of agricultural products for numerous diseases
which are (thankfully)

extremely rare, such as E. coli.

12. The USDA has performed BSE tests on thousands of cattle younger than 30

months, including thousands that had no clinical signs of BSE.

13. Measures that the United States (and Canada) has put in place to
reduce the

transmission of BSE have substantially reduced the risk in the United
States, but have not

eliminated it. Experience has shown that the feed bans are incomplete,
both because the bans are

limited and because of imperfect implementation.

14. Collecting additional BSE test data from cattle less likely to have
detectable levels

of BSE (because they are younger and do not display other risk factors),
while not as cost-

effective, is not worthless and produces data that could help better
understand BSE and its

distribution in U.S. cattle. (For example, if authorities only tested
cattle suspected of having

BSE, the Canadians would not have found BSE in the 50 month old Alberta
cow that did not

have outward signs that might have suggested BSE and was born long after
exposure to BSE in

-7


Case 1:06-cv-00544-JR Document 14-9 Filed 11/03/2006 Page 9 of 47

cattle feed was believed to have ceased; nor is it likely that either of
the two indigenously

infected US cows with atypical BSE that tested positive would have been
identified.) Evolving

knowledge about atypical BSE, where there are only rarely outward signs
that might cause the

animal to be singled out and tested based on suspicion of BSE, makes the
value of testing

asymptomatic cattle even clearer. Voluntary testing does not interfere
with or dilute government

efforts to test for BSE and to understand the disease. Additionally,
while most cattle slaughtered

in the U.S. are under 30 months of age, many thousands every year are
over 30. I am not aware

of any other programs under which USDA has prohibited testing for a
disease because it is

unlikely that the disease will be found.

15. The USDA recently announced a dramatic (ten-fold) reduction in the
number of

cattle it will test for BSE. USDA plans to reduce the current annual
rate of over 400,000 cows to

only 40,000 (less than 1 % of the cattle that are slaughtered or die
each year). USDA News

Release No. 0255.06, July 20, 2006, available at

http://www.usda.gov/wps/portal/!ut/p/_s.7_0_A/7_0_1OB?contentidonly=true&contentid=2006/


07/0255.xml. Preventing voluntary testing while at the same time
dramatically reducing

government testing does not make any sense for the protection of animal
and human health and

is further indication that voluntary BSE testing is not "worthless."
Although the USDA and the

OIE have stated that targeted testing of cattle most likely to have BSE
is the most effective way

of detecting and monitoring BSE infection in a country's cattle herd,
most developed countries

known to have BSE nonetheless have chosen to go far beyond such targeted
testing in their

domestic BSE monitoring programs, especially in view of the growing
proportion of infected

cattle that have atypical (and asymptomatic) forms of BSE when slaughtered.

-8


Case 1:06-cv-00544-JR Document 14-9 Filed 11/03/2006 Page 10 of 47

16. BSE tests do not involve the immune system of the animal that is
tested. There is

a sharp distinction between using immunological means to prevent foreign
pathogen infections

(e.g., active and passive immunization) and the use of an immunologic
reagent (e.g., an

antibody) to detect infectious prion proteins obtained from the body of
an infected host. The use

of such a test has nothing whatsoever to do with the immune system of
the infected (and now

dead) animal. USDA thinking on this point is very confused.

17. The USDA allows suppliers of meat and other products to certify that
those

products meet criteria unrelated to food safety (or least, where USDA
does not have data to

conclude that there is a food safety benefit). I believe this is true,
for example, of "organic,

"natural," "hormone-free, "grass-fed," and other descriptors that USDA
allows. Given the

legitimate role of the USDA to protect consumers from industry
incompetence, it nevertheless

seems unreasonable for USDA to prohibit the private use of its own
validated test method. The

desire on the part of some consumers to have a higher standard of safety
with respect to what

they eat should not be circumvented by this kind of attitude, even if
most people are satisfied

with government assurances of safety based on its own evaluation.

I declare under penalty of perjury that the foregoing is true and correct.

Executed on October 27, 2006. Paul Brown, M.D.

-9


Case 1:06-cv-00544-JR Document 14-9 Filed 11/03/2006 Page 11 of 47

ATTACHMENT A

Paul W. Brown, M.D.

List of Publications 1991 to Present


Case 1:06-cv-00544-JR Document 14-9 Filed 11/03/2006 Page 12 of 47

Paul W. Brown, M.D.

LIST OF PUBLICATIONS 1991 TO PRESENT

153. Goldfarb, L.G., Brown, P. and Gajdusek, D.C. Multiple mutations in

kuru, Creutzfeldt-Jakob disease, and Gerstmann-Sträussler syndrome.

Brain Research, 16: 1 (January), 98-99, 1991.

154. Brown P. and Gajdusek, D.C. New studies on the resistance of scrapie

agent to enzymatic digestion, heat and chemical denaturation, and

natural weathering. Brain Research, 16: 1 (January), 100-103, 1991.

155. Brown, P. and Gajdusek, D.C. Survival of scrapie virus after 3 years’

interment. Lancet, 337: 8736 (February 2), 269-270, 1991.

156. Goldfarb L.G., Haltia, M., Brown, P., Nieto, A., Kovanan, J.,

McCombie, W.R., Trapp, S. and Gajdusek, D.C. New mutation in scrapie

amyloid precursor gene (codon 178) in Finnish Creutzfeldt-Jakob

kindred. Lancet, 337: 8738 (February 16), 425, 1991.

157. Nieto, A., Goldfarb, L.G., Brown, P., McCombie W.R., Trapp, S.,

Asher, D.M. and Gajdusek, D.C. Codon 178 mutation in ethnically

diverse Creutzfeldt-Jakob disease families. Lancet, 337: 8741

(March 9), 622-623, 1991.

158. Fradkin, J.E., Schonberger, L., Mills, J.L., Gunn, W.J., Piper, J.M.,

Wysowski, D.K., Thomson, R., Durako, S. and Brown, P. Creutzfeldt-

Jakob disease in pituitary growth hormone recipients in the United

States. Journal of the American Medical Association, 265: 7

(February 20), 880-884, 1991. Also as abstract:

Fradkin, J.E., Schonberger, L., Mills, J.L., Gunn, W.J., Piper, J.M.,

Wysowski, D.K., Thomson, R., Durako, S. and Brown, P. Creutzfeldt-

Jakob disease in pituitary growth hormone recipients in the United

States. In: “Program and Abstracts, The Endocrine Society 72nd

Annual Meeting, Atlanta, GA, June 20-23, 1990, p. 350.

159. Brown, P., Goldfarb, L.G., Brown, W.T., Goldgaber, D., Rubenstein, R.,

Kascsak, R. J., Piccardo, P., Boellaard, J.W. and Gajdusek, D.C.

Clinical and molecular genetic study of a large German kindred with

Gerstmann-Sträussler-Scheinker syndrome. Neurology, 41: 3 (March),

375-379, 1991.

160. Brown, P., Goldfarb, L.G. and Gajdusek, D.C. The new biology of

spongiform encephalopathy: infectious amyloidoses with a genetic

twist. Lancet, 337: 8748 (April 27), 1019-1022, 1991.

161. Pocchiari, M., Peano, S., Conz, A., Eshkol, A., Maillard, F., Brown,

P., Gibbs, C.J., Jr., Geng Xi, Y., Tenham-Fisher, E. and Macchi, G.

Combination ultrafiltration and 6 M urea treatment of human growth

hormone effectively minimizes risk from potential Creutzfeldt-Jakob

disease virus contamination. Hormone Research, 35: 3-4

(March-April), 161-166, 1991.


Case 1:06-cv-00544-JR Document 14-9 Filed 11/03/2006 Page 13 of 47

1991 (Con’t.)

162. Brown, P., Goldfarb, L.G., Gibbs, C.J., Jr. and Gajdusek, D.C.

The phenotypic expression of different mutations in transmissible

familial Creutzfeldt-Jakob disease. European Journal of

Epidemiology, 7:5 (September), 469-476, 1991.

163. Goldfarb, L.G., Brown, P., Mitrová, E., Haltia, M., Cervenáková, L.,

Goldin, L., Korczyn, A., Chapman, J., Galvez, S., Cartier, L.,

Rubenstein, R. and Gajdusek, D.C. Familial Creutzfeldt-Jakob disease

associated with the PRNP codon 200Lys mutation: an analysis of 45

families. European Journal of Epidemiology, 7:5 (September), 477486, 1991.

164. Mitrová, E., Brown, P., Hroncová, D., Tatara, M. and Zilák, J. Focal

accumulation of CJD in Slovakia: retrospective investigation of a new

rural familial cluster. European Journal of Epidemiology, 7:5

(September), 487-489, 1991.

165. Haltia, M., Kovanen, J., Goldfarb, L.G., Brown, P. and Gajdusek, D.C.

Familial Creutzfeldt-Jakob disease in Finland: epidemiological,

clinical, pathological and molecular genetic studies. European

Journal of Epidemiology, 1991, 7:5 (September), 494-500, 1991.

Also as abstracts:

Haltia, M., Kovanen, J., Goldfarb, L.G. and Gajdusek, D.C. Novel

mutation in the PRNP amyloid precursor gene co-segregates with

Creutzfeldt-Jakob disease in a Finnish family. Abstracts of the 36th

Annual Meeting of the Deutsche Gesellschaft für Neuropathologie und

Neuroanatomie Düsseldorf, September 16-18, 1991. Clinical

Neuropathology, 10: 5 (September/October), 257, 1991

Haltia, M., Kovanen, J., Goldfarb, L.G., Brown, P. and Gajdusek, D.C.

A new mutation (at codon 178) in the PRNP amyloid precursor gene
cosegregates with Creutzfeldt-Jakob disease in a large Finnish kindred.

Abstracts of the First Hungarian Conference of Neuropathology and the

5th Hungarian-Polish Neuropathological Symposium, Budapest, September

26-28, 1991. Ideggyógyászati Szemle (Neurological Review), 44

(Suppl. 1), 35, 1991.

166. Liberski, P.P., Kwiecinski, H., Barcikowska, M., Mirecka, B.,

Kulczycki, J., Kida, E., Brown, P. and Gajdusek, D.C. PrP amyloid

plaques in Creutzfeldt-Jakob disease of short duration:

immunohistochemical studies of 5 cases from Poland. European Journal

of Epidemiology, 7:5 (September), 505-510, 1991.

167. Brown, P. The clinical epidemiology of Creutzfeldt-Jakob disease in

the context of bovine spongiform encephalopathy. In: “Sub-acute

Spongiform Encephalopathies”, R. Bradley, M. Savey and B.A.

Marchant, editors. Kluwer Academic Publishers, Dordrecht

(The Netherlands), 1991, pp.195-202.

168. Brown, P. and Gajdusek, D.C. The human spongiform encephalopathies:

kuru, Creutzfeldt-Jakob disease, and the Gerstmann-Sträussler-

Scheinker syndrome. In: “Transmissible Spongiform Encephalopathies:

Scrapie, BSE and Related Disorders”, B.W. Chesebro, editor. Current

Topics in Microbiology and Imunology, volume 172, Springer Verlag,

Berlin, 1991, pp.1-20.

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Case 1:06-cv-00544-JR Document 14-9 Filed 11/03/2006 Page 14 of 47

1991 (Con’t.)

169. Brown, P. Molecular genetics of spongiform encephalopathy.

Neuroscience Facts, 2:19 (October 3), 2, 1991.

170. Brown, P., Goldfarb, L.G., Cathala, F., Vrbovská, A., Sulima, M.,

Nieto, A., Gibbs, C.J., Jr. and Gajdusek, D.C. The molecular

genetics of familial Creutzfeldt-Jakob disease in France. Journal

of the Neurological Sciences, 105:2 (October), 240-246, 1991.

171. Scrimgeour, E.M. and Brown, P. BSE and potential risks to

slaughtermen. Veterinary Record, 129: 17 (October 26), 390-391,

1991.

172. Liberski, P.P., Brown, P., Shu-Yan, X. and Gajdusek, D.C. The

ultrastructural diversity of scrapie-associated fibrils isolated

from experimental scrapie and Creutzfeldt-Jakob disease. Journal

of Comparative Pathology, 105:4 (November), 377-386, 1991.

173. Trabattoni, G., Lechi, A., Bettoni, L., Macchi, G., Masullo, C.,

Brown, P. and Pocchiari, M. Creutzfeldt-Jakob disease in Italy

(letter to the editor). European Journal of Epidemiology, 7: 6

(November), 713-714, 1991.

174. Goldfarb, L.G., Brown, P., McCombie, W.R., Goldgaber, D.,

Swergold, G.D., Wills, P.R., Cervenáková, L., Baron, H.,

Gibbs, C.J., Jr. and Gajdusek, D.C. Transmissible familial

Creutzfeldt-Jakob disease associated with five, seven, and eight

extra octapeptide coding repeats in the PRNP gene. Proceedings of

the National Academy of Sciences (USA), 88:23 (December 1), 1092610930,
1991.

175. Laplanche, J.-L., Chatelain, J., Thomas, S., Brown, P. and Cathala, F.

Analyse du gene PrP dans une famille d’origine Tunisienne atteinte de

maladie de Creutzfeldt-Jakob. Revue Neurologique (Paris), 147: 12

(December), 825-827, 1991.

176. Korczyn, A.D., Chapman, J., Goldfarb, L.G., Brown, P. and Gajdusek,

D.C. A mutation in the prion protein gene in Creutzfeldt-Jakob

disease in Jewish patients of Libyan, Greek, and Tunisian origin.

Annals of the New York Academy of Sciences, 640:(December 3), 171176, 1991.

177. Brown, P., Goldfarb, L.G., McCombie, W.R., Nieto, A., Squillacote, D.,

Sheremata, W., Little, B.W., Godec, M.S., Gibbs, C.J., Jr. and

Gajdusek, D.C. Atypical Creutzfeldt-Jakob disease in an American

family with an insert mutation in the PRNP amyloid precursor gene.

Neurology, 42:2 (February), 422-427, 1992.

178. Goldfarb, L.G., Brown, P., Haltia, M., Cathala, F., McCombie, W.R.,

Kovanen, J., Goldin, L., Nieto, A., Godec, M.S., Asher, D.M.and

Gajdusek, D.C. Creutzfeldt-Jakob disease co-segregates with the

codon 178Asn PRNP mutation in families of European origin.

Annals of Neurology, 31:3 (March), 274-281, 1992.

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1992 (Con’t.)

179. Brown, P., Goldfarb, L.G., Kovanen, J., Haltia, M., Cathala, F.,

Sulima, M., Gibbs, C.J., Jr. and Gajdusek, D.C. Phenotypic

characteristics of familial Creutzfeldt-Jakob disease associated with

the codon 178Asn mutation. Annals of Neurology, 31:3 (March), 282-285,

1992.

180. Brown, P. The phenotypic expression of different mutations in

transmissible human spongiform encephalopathy. Revue Neurologique

(Paris), 148:5 (May), 317-327, 1992.

181. Chapman, J., Brown, P., Rabey, M.J., Goldfarb, L.G., Inzelberg, R.,

Gibbs, C.J., Jr., Gajdusek, D.C. and Korczyn, A.D. Transmission of

spongiform encephalopathy from a familial Creutzfeldt-Jakob disease

patient of Jewish Libyan origin carrying the PRNP codon 200 mutation.

Neurology, 42:6 (June), 1249-1250. 1992. Also as abstract:

Brown, P., Goldfarb, L.G., Gibbs, C.J., Jr., Gajdusek, D.C.,

Chapman, J., Rabey, M.J., Inzelberg, R. and Korczyn, A.D.

Transmission of spongiform encephalopathy from a Creutzfeldt-Jakob

disease (CJD) patient of Jewish Libyan origin carrying the PRNP

codon 200 mutation. Abstract no. 781S in: “ American Academy of

Neurology 44th Annual Meeting”, San Diego, May 3-9, 1992.

Neurology, 42:4 (April), Supplement 3, 370, 1992.

182. Brown, P., Preece, M.A. and Will, R.G. “Friendly fire” in medicine:

hormones, homografts, and Creutzfeldt-Jakob disease. Lancet,

340:8810 (July 4), 24-27, 1992.

183. Goldfarb, L.G., Brown, P., Vrbovská, A., Baron, H., McCombie, W.R.,

Cathala, F., Gibbs, C.J., Jr. and Gajdusek, D.C. An insert mutation

in the chromosome 20 amyloid precursor gene in a Gerstmann-

Sträussler-Scheinker family. Journal of the Neurological Sciences,

111:2 (September), 189-194, 1992.

184. Brown, P., Gálvez, S., Goldfarb, L.G., Nieto, A., Cartier, L.,

Gibbs, C.J., Jr. and Gajdusek, D.C. Familial Creutzfeldt-Jakob

disease in Chile is associated with the codon 200 mutation of the

PRNP amyloid precursor gene on chromosome 20. Journal of the

Neurological Sciences, 112:1,2 (October), 65-67, 1992.

185. Goldfarb, L.G., Petersen, R.B., Tabaton, M., Brown, P, LeBlanc, A.C.,

Montagna, P., Cortelli, P., Julien, J., Vital, C., Pendelbury, W.W.,

Haltia, M., Wills, P.R, Hauw, J.J., McKeever, P.E., Monari, L.,

Schrank, B., Swergold, G.D., Autilio-Gambetti, L., Gajdusek, D.C.,

Lugaresi, E. and Gambetti, P. Fatal familial insomnia and familial

Creutzfeldt-Jakob disease: disease phenotype determined by a DNA

polymorphism. Science, 258:5083 (October 30), 806-808, 1992.

Also as abstract:

Petersen, R.B., Goldfarb, L., Tabaton, M., Brown, P, LeBlanc, A.,

Montagna, P., Cortelli, P., Monari, L., Autilio-Gambetti, L.,

Gajdusek, D.C., Lugaresi, E. and Gambetti, P. Fatal familial

insomnia and one subtype of familial Creutzfeldt-Jakob disease:

effect of a polymorphism on a pathogenic mutation in the prion

protein. The FASEB Journal, 7: 4 (February 23), A627, 1993.

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1992 (Con’t.)

186. Bertoni, J.M., Brown, P., Goldfarb, L.G., Rubenstein, R. and

Gajdusek, D.C. Familial Creutzfeldt-Jakob disease (codon 200

mutation) with supranuclear palsy. Journal of the American Medical

Association, 268:17 (November 4), 2413-2415, 1992.

187. Goldfarb, L.G., Brown, P. and Gajdusek, D.C. The molecular genetics of

human transmissible spongiform encephalopathy. In: “Prion Diseases

of Humans and Animals”, S.B. Prusiner, J. Collinge, J. Powell and

B. Anderton, editors, Ellis Horwood, Chichester (England), 1992,

pp.139-153.

1993

188. Brown, P. The molecular biology and genetics of spongiform

encephalopathy. In: “Light and Electron Microscopic Neuropathology

of Slow Virus Disorders”, P.P. Liberski, editor. CRC Press, Boca

Raton, Florida, 1993, pp.63-100.

189. Liberski, P.P., Guiroy, D.C., Williams, E.S., Yanagihara, R.,
Brown, P.

and Gajdusek, D.C. The amyloid plaque. In: “Light and Electron

Microscopic Neuropathology of Slow Virus Disorders”, P.P. Liberski,

editor. CRC Press, Boca Raton, Florida, 1993, pp.295-347.

190. Liberski, P.P. and Brown, P. Scrapie-associated fibrils. In: “Light

and Electron Microscopic Neuropathology of Slow Virus Disorders”,

P.P. Liberski, editor. CRC Press, Boca Raton, Florida, 1993, pp.393414.

191. Brown, P., Kaur, P., Sulima, M.P., Goldfarb, L.G., Gibbs, C.J., Jr.
and

Gajdusek, D.C. Real and imagined clinicopathological limits of

“prion dementia”. Lancet, 341:8838 (January 16), 127-129, 1993.

192. Brown, P. “Prion dementia”: Author’s reply to Letters to the Editor

concerning Real and imagined clinicopathological limits of “prion

dementia”. Lancet, 341:8845 (March 6), 627-628, 1993.

193. Gibbs, C.J., Jr., Asher, D.M., Brown, P.W., Fradkin, J.E. and
Gajdusek,

D.C. Creutzfeldt-Jakob disease infectivity of growth hormone derived

from human pituitary glands. New England Journal of Medicine, 328:5

(February 4), 358-359, 1993.

194. Goldfarb, L.G., Brown, P., Haltia, M., Ghiso, J., Frangione, B. and

Gajdusek, D.C. Synthetic peptides corresponding to different mutated

regions of the amyloid gene in familial Creutzfeldt-Jakob disease

show enhanced in vitro formation of morphologically different amyloid

fibrils. Proceedings of the National Academy of Sciences (USA),

90:10 (May 15), 4451-4454, 1993.

195. Brown, P. EEG findings in Creutzfeldt-Jakob disease (Questions and

Answers section). Journal of the American Medical Association, 269:

24 (June 23), 3168, 1993.

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1993 (Con’t.)

196. Brown, P. Infectious cerebral amyloidosis: clinical spectrum, risks

and remedies. In: “Developments in Biological Standardization”,

F. Brown, editor. Karger, Basal, 1993, volume 80, pp.91-101.

Also as abstract:

Brown, P. The clinico-pathologic features of transmissible human

spongiform encephalopathy, with a discussion of recognized risk

factors and preventive strategies. In: Abstracts of an International

Meeting on Transmissible Spongiform Encephalopathies: Impact on

Animal and Human Health, Heidelberg, June 23-24, 1992, p.4.

197. Goldfarb, L.G., Brown, P., Little, B.W., Cervenáková, L., Kenney, K.,

Gibbs, C.J., Jr. and Gajdusek, D.C. A new (two repeat) octapeptide

coding insert mutation in Creutzfeldt-Jakob disease. Neurology, 43:

11 (November), 2392-2394, 1993.

198. Barcikowska, M., Liberski, P.P, Boellaard, J.W., Brown, P.,

Gajdusek, D.C. and Budka, H. Microglia is a component of the

prion protein amyloid plaque in the Gerstmann-Sträussler-Scheinker

syndrome. Acta Neuropathologica, 85: 6 (May), 623-627, 1993.

199. Chapman, J., Brown, P., Goldfarb, L.G., Arlazoroff, A., Gajdusek, D.C.

and Korczyn, A.D. Clinical heterogeneity and unusual presentations

of Creutzfeldt-Jakob disease in Jewish patients with the PRNP codon

200 mutation. Journal of Neurology, Neurosurgery and Psychiatry, 56:

10 (October), 1109-1112, 1993.

1994

200. Brown, P. Transmissible human spongiform encephalopathy

(infectious cerebral amyloidosis): Creutzfeldt-Jakob disease,

Gerstmann-Sträussler-Scheinker syndrome, and kuru. Chapter 48

in: “Neuro-degenerative Diseases”, D.B. Calne, editor.

W.B. Saunders Co., Philadelphia, 1994, pp.839-876.

201. Brown, P. Infectious cerebral amyloidoses: Creutzfeldt-Jakob disease

and the Gerstmann-Sträussler-Scheinker syndrome. Chapter 14 in:

“Handbook of Dementing Illnesses”, J C. Morris, editor. Marcel

Dekker, Inc., New York, 1994, pp.353-375.

Also as abstract:

Brown, P. Clinical and laboratory diagnosis of spongiform

encephalopathy. XIVth European Congress of Pathology, September

5-10, 1993, Innsbruck, Austria. Pathology Research and Practice,

189: 6-7 (August), 661, 1993.

202. Brown, P., Cervenáková, L., Goldfarb, L.G., Gajdusek, D.C.,

Haverkamp, A., Haverkamp, C., Horwitz, J., Creacy, S.D., Bever, R.A.,

Wexler, P., Sujansky, E. and Bjork, R.J. Molecular genetic testing of a

fetus at risk of Gerstmann-Sträussler Scheinker syndrome. Lancet, 343:

8890 (January 15), 181-182, 1994.

203. Goldfarb, L.G., Brown, P., Cervenáková, L. and Gajdusek, D.C.

Genetic analysis of Creutzfeldt-Jakob disease and related disorders.

Philosophical Transactions of the Royal Society of London (Series B),

343: 1306 (March 29), 379-384, 1994.

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1994 (Con’t.)

204. Brown, P., Goldfarb, L.G., Cervenáková, L., McCombie, W.R.,

Rubenstein, R., Will, R.G., Pocchiari, M., Martinez-Lage, J.F., Scalici,

C., Masullo, C, Graupera, G., Ligan, J. and Gajdusek, D.C. Iatrogenic

Creutzfeldt-Jakob disease: an example of the interplay between ancient

genes and modern medicine. Neurology, 44: 2 (February), 291-293, 1994.

205. Monari, L., Chen, S.G., Brown, P., Parchi, P., Petersen, R.B.,

Mikol, J., Gray, F., Cortelli, P., Montagna, P., Ghetti, B., Goldfarb,

L.G., Gajdusek, D.C., Lugaresi, E., Gambetti, P. and Autilio-Gambetti,

L. Fatal familial insomnia and familial Creutzfeldt-Jakob disease:

different prion proteins determined

by a DNA polymorphism. Proceedings of the National Academy of Sciences

(USA), 91: 7 (March 29), 2839-2842, 1994.

206. Lane, K.L., Brown, P., Howell, D.N., Crain, B.J., Hulette, C.M.,

Burger, P.C. and DeArmond, S.J. Creutzfeldt-Jakob disease in a pregnant

woman with an implanted dura mater graft. Neurosurgery,

34: 4 (April), 737-740, 1994.

207. Alperovitch, A., Brown, P., Weber, T., Pocchiari, M., Hofman, A.
and Will,

R. Incidence of Creutzfeldt-Jakob disease in Europe in 1993. Lancet,

343: 8902 (April 9), 918, 1994.

208. Garruto, R.M. and Brown, P. Tau protein, aluminium, and Alzheimer’s

disease (Commentary). Lancet, 343: 8904 (April 23), 989, 1994.

209. Brown, P., Gibbs, C.J., Jr., Rodgers-Johnson, P., Asher, D.M.,

Sulima, M.P., Bacote, A., Goldfarb, L.G. and Gajdusek, D.C.

Human spongiform encephalopathy: the National Institutes of

Health Series of 300 cases of experimentally transmitted disease.

Annals of Neurology, 35: 5 (May), 513-529, 1994.

210. Brown, P. The “brave new world” of transmissible spongiform

encephalopathy (infectious cerebral amyloidosis). Molecular

Neurobiology, 8: 2-3 (April-June), 79-87, 1994.

211. Goldfarb, L.G., Brown, P., Cervenáková, L. and Gajdusek, D.C.

Molecular genetic studies of Creutzfeldt-Jakob disease.

Molecular Neurobiology, 8: 2-3 (April-June), 89-97, 1994.

212. Petersen, R.B., Goldfarb, L.G., Tabaton, M., Brown, P., Monari, L.,

Cortelli, P., Montagna, P., Autilio-Gambetti, L., Gajdusek, D. C.,

Lugaresi, E. and Gambetti, P. A novel mechanism of phenotypic

heterogeneity demonstrated by the effect of a polymorphism on a

pathogenic mutation in the PRNP (prion protein gene). Molecular

Neurobiology, 8: 2-3 (April-June), 99-103, 1994.

213. Brown, P., Cervenáková, L., Boellaard, J.W., Stavrou, D., Goldfarb,
L.G.

and Gajdusek, D.C. Identification of a PRNP mutation in Jakob’s

original Creutzfeldt-Jakob disease family. Lancet, 344: 8915

(July 9), 130-131, 1994.

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1994 (Con’t.)

214. Haltia, M., Viitanen, M., Sulkava, R., Ala-Hurula, V., Poyhonen, M.,

Goldfarb, L., Brown, P., Levy, E., Houlden, H., Crook, R., Goate, A.,

Clark, R., Korenblat, K., Pandit, S., Keller, H.D., Lilius, L.,

Liu, L., Axelman, K., Forsell, L., Winblad, B., Lannfelt, L. and Hardy,

J. Chromosome 14-encoded Alzheimer’s disease: genetic and

clinicopathological description. Annals of Neurology, 36: 3

(September), 362-367, 1994.

215. Brown, P. [Debate] Vertical transmission of prion disease [response

to an article entitled “The transmission of prion disease: vertical

transfer of prion disease” by R.W. Lacey and S.F. Dealler]. Human

Reproduction, 9: 10 (October), 1796-1797, 1994.

216. Martinez-Lage, J.F., Poza, M., Sola, J., Tortosa, J.G., Brown, P.,

Cervenáková, L., Esteban, J.A. and Mendoza, A. Accidental transmission

of Creutzfeldt-Jakob disease by dural cadaveric grafts. Journal of

Neurology, Neurosurgery and Psychiatry, 57: 10 (October), 1091-1094,

1994.

217. Cervenáková, L., Brown, P., Goldfarb, L.G., Nagle, J., Pettrone, K.,

Rubenstein, R., Gibbs, C.J., Jr and Gajdusek, D.C. Infectious amyloid

precursor gene sequences in primates used for experimental transmission

of human spongiform encephalopathy. Proceedings of the National Academy

of Sciences (USA), 91, 25, (December 6), 12159-12162, 1994.

1995

218. Goldfarb, L.G. and Brown, P. The transmissible spongiform

encephalopathies. Annual Review of Medicine, 46: 57-65, 1995.

219. Lang, C.J.G., Schüler, P., Engelhardt, A., Spring, A. and Brown, P.

Probable Creutzfeldt-Jakob disease after a cadaveric dural graft.

European Journal of Epidemiology, 11: 1 (February), 79-81, 1995.

220. Billette de Villemeur, T., Fournier, J-G., Robain, O., Escaig-Haye,
F. and

Brown, P. Electronmicroscopic detection of prion-protein-positive

fibres in brain from iatrogenic Creutzfeldt-Jakob disease. Lancet, 345:

8953 (April 1), 861-862, 1995.

221. Barcikowska, M., Kwiecinski, H., Liberski, P.P., Kowalski, J.,

Brown, P. and Gajdusek, D.C. Creutzfeldt-Jakob disease with Alzheimer-

type Ab-reactive amyloid plaques. Histopathology,

26:5 (May), 445-450, 1995.

222. Reder, A.T., Mednick, A.S., Brown, P., Spire, J.P., Van Cauter, E.,

Wollmann, R.L., Cervenáková, L., Goldfarb, L.G., Garay, A.,

Ovsiew, F., Gajdusek, D.C. and Roos, R.P. Clinical and genetic studies

of fatal familial insomnia. Neurology, 45: 6 (June),

1068-1075, 1995. Also as abstract:

Mednick, A.S., Reder, T., Spire, J.P., Van Cauter, E., Brown, P.,

Wollmann, R.L., Goldfarb, L.G., Garay, A., Ovsiew, F., Gajdusek, D.C.

and Roos, R.P. Fatal familial insomnia (FFI). Proceedings of the 46th

Annual Meeting of the American Academy of Neurology, May 1-7, 1994 in

Washington, D.C. (Abstract 629P). Neurology, 44: (Suppl 2)

4 (April), A285-286, 1994.

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1995 (Con’t.)

223. Hainfellner, J.A., Brantner-Inthaler, S., Cervenáková, L., Brown, P.,

Kitamoto, T., Tateishi, J., Diringer, H., Liberski, P.P., Regele, H.,

Feucht, M., Mayr, N., Wessely, P., Summer, K., Seitelberger, F. and

Budka, H. The original Gerstmann-Stäussler-Scheinker family of Austria:

divergent clinicopathological phenotypes but constant PrP genotype.

Brain Pathology, 5: 3 (July), 201-211, 1995.

224. Budka, H., Aguzzi, A., Brown, P., Brucher, J-M., Bugiani, O.,

Collinge, J., Diringer, H., Gullotta, F., Haltia, M., Hauw, J-J.,

Ironside, J.W., Kretzschmar, H.A., Lantos, P.L., Masullo, C., Pocchiari,

M., Schlote, W., Tateishi, J. and Will, R.G. Consensus Report: Tissue

handling in suspected Creutzfeldt-Jakob disease (CJD) and other human

spongiform encephalopathies (prion diseases). Brain Pathology, 5: 3

(July), 319-322, 1995.

225. Budka, H., Aguzzi, A., Brown, P., Brucher, J-M., Bugiani, O.,

Gullotta, F., Haltia, M., Hauw, J.-J., Ironside, J.W., Jelinger, K.,

Kretzschmar, H.A., Lantos, P.L., Masullo, C., Schlote, W.,

Tateishi, J. and Weller, R.O. Consensus Report: Neuropathological

diagnostic criteria for Creutzfeldt-Jakob disease (CJD) and other human

spongiform encephalopathies (prion diseases). Brain Pathology, 5: 3

(July), 459-466.

226. Tateishi, J., Brown, P., Kitamoto, T., Hoque, Z.M., Roos, R.,

Wollman, R. and Gajdusek, D.C. First experimental transmission

of fatal familial insomnia. Nature, 376: 6539 (August 3), 434-435,

1995.

227. Brown, P., Kenney, K., Little, B., Ironside, J., Will, R.,

Cervenáková, L., San Martin, R.A., Safar, J., Roos, R., Harris, S.,

Haltia, M., Gibbs, C.J., Jr. and Gajdusek, D.C. Intracerebral

distribution of infectious amyloid protein in spongiform encephalopathy.
Annals of Neurology, 38: 2 (August), 245-253, 1995.

Also as abstract:

Brown, P., Kenney, K., Little, B., Ironside, J., Safar, J., Rohwer, R.,

Roos, R., Wollmann, R., Gibbs, C.J., Jr and Gajdusek, D.C. Comparison

of clinical features, neuropathology, and intracerebral distribution of

PrP amyloid protein in the brains of patients with spongiform

encephalopathy. Neurobiology of Aging, 15 (Supplement 1), p.S150

(Abstract No. 619), 1994.

228. Beekes, M., Baldauf, E., Cassens, S., Diringer, H., Keyes, P.,

Scott, A.C., Wells, G.A.H., Brown, P., Gibbs, C.J., Jr. and

Gajdusek, D.C. Western blot mapping of disease-specific amyloid

in various animal species and humans with transmissible spongiform

encephalopathies using a high-yield purification method.

Journal of General Virology, 76: 10 (October), 2567-2576, 1995.

229. Brown, P. Can Creutzfeldt-Jakob disease be transmitted by transfusion?

Current Opinion in Hematology, 2: 6 (November), 472-477, 1995.

230. Brown, P. [Education and Debate] Creutzfeldt-Jakob disease and bovine

spongiform encephalopathy: any connection? The jury is still out.

British Medical Journal, 311: 7017 (November 25), 1416, 1995.

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Case 1:06-cv-00544-JR Document 14-9 Filed 11/03/2006 Page 21 of 47

231. Masullo, C., Brown, P.W. and Macchi, G. Creutzfeldt-Jakob disease

in an Iranian: the first clinico-pathologically described case.

Clinical Neuropathology, 15: 1 (January), 26-29, 1996.

232. Budka, H., Aguzzi, A., Brown, P., Brucher, J.-M., Bugiani, O.,

Collinge, J., Diringer, H., Gullotta, F., Haltia, M., Hauw, J.-J.,

Ironside, J.W., Kretzschmar, H.A., Lantos, P.L., Masullo, C.,

Pocchiari, M., Schlote, W., Tateishi, J. and Will, R.G.

Konsensusbericht: gewebsbehandlung bei verdacht auf Creutzfeldt-Jakob-

Krankheit und andere spongiforme enzephalopathien (prionen-krankeiten)

des menschen. Pathologe, 17: 2 (February), 171-176, 1996.

233. Chapman, J., Arlazoroff, A., Goldfarb, L.G., Cervenáková, L., Neufeld,

M.Y., Werber, E., Herbert, M., Brown, P., Gajdusek, D.C. and Korczyn,

A.D. Fatal insomnia in a case of familial Creutzfeldt-Jakob disease

with the codon 200Lys mutation. Neurology, 46: 3 (March), 758-761,

1996.

234. Liberski, P., Yanagihara, R., Brown, P., Kordek, R., Kloszewska, I.,

Bratosiewicz., J. and Gajdusek, D.C. Microwave treatment enhances the

immunostaining of amyloid deposits in both the transmissible

and non-transmissible brain amyloidoses. Neurodegeneration, 5: 1

(March), 95-99, 1996.

235. Brown, P. Bovine spongiform encephalopathy and Creutzfeldt-Jakob
disease:

the link is unproved but no better explanation is presently

forthcoming. British Medical Journal, 312: 7034 (March 30), 790-791,

1996.

236. Salvatore, M., Galvez, S., Brown, P., Macchi, G., Fieschi, C.,

Cardone, F., Petraroli, R., Colosimo, C., D'Allessandro, M. and

Pocchiari, M. Codon 200 mutation in a new Creutzfeldt-Jakob disease

family of Chilean origin. Journal of Neurology, Neurosurgery, and

Psychiatry, 61: 1 (July), 111-112, 1996.

237. Brown, P. Environmental causes of human spongiform encephalopathy.

In: “Methods in Molecular Medicine: Prion diseases", H. Baker and

R. Ridley, editors, Humana Press, Totowa, New Jersey, pp.139-154, 1996.

238. Brown, P. Transmissible cerebral amyloidosis. Journal of Neural

Transmission, Supplement 47, 219-229, 1996.

239. Scrimgeour, E.M., Brown, P and Monaghan, P. Disposal of rendered

specified offal. Veterinary Record, 139: 9 (August 31), 219-220, 1996.

240. Cochran, E.J., Bennett, D.A., Cervenáková, L., Kenney, K., Bernard,
B.,

Foster, N.L., Benson, D.F., Goldfarb, L.G. and Brown, P. Familial

Creutzfeldt-Jakob disease with a five-repeat octapeptide insert

mutation. Neurology, 47: 9 (September), 727-733, 1996.

241. Scrimgeour, E.M., Chand, P.R., Kenney, K. and Brown, P.
Creutzfeldt-Jakob

disease in Oman: report of two cases. Journal of the Neurological

Sciences, 142: 1-2 (October), 148-150, 1996.

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1996 (Con’t.)

242. Will, R.G., Zeidler, M., Brown, P., Harrington, M., Lee, K.H. and

Kenney, K.L. Cerebrospinal-fluid test for new-variant Creutzfeldt-Jakob

disease. Lancet, 348: 9032 (October 5), 955, 1996.

243. Silburn, P., Cervenáková, L., Varghese, P., Tannenberg, A., Brown,
P. and

Boyle, R. Fatal familial insomnia -a seventh family. Neurology, 47: 5

(November), 1326-1328, 1996.

244. El Hachimi, K.H., Cervenáková, L., Brown, P., Goldfarb, L.G.,
Rubenstein,

R., Gajdusek, D.C. and Foncin, J.-F. Mixed features of Alzheimer

disease and Creutzfeldt-Jakob disease in a family with a presenilin-1

mutation in chromosome 14. Amyloid, 3: 4 (December), 223-233, 1996.

245. Goldfarb, L.G., Cervenáková, L., Brown, P. and Gajdusek, D.C.

Genotype-phenotype correlations in familial spongiform

encephalopathies associated with insert mutations. In:

"Transmissible Subacute Spongiform Encephalopathies: Prion Diseases".

(Proceedings of the IIIrd International Symposium on Transmissible

Subacute Spongiform Encephalopathies: Prion Diseases, 18-20 March

1996, Val de Grâce, Paris, France). L. Court and

B. Dodet, editors, Elsevier, Amsterdam, 1996 pp.425-431.

246. Cervenáková, L., Brown, P., Piccardo, P., Cummings, J.L., Nagle, J.,

Vinters, H.V., Kaur, P., Ghetti, B., Gajdusek, D.C. and Goldfarb,

L.G. 24-nucleotide deletion in the PRNP gene: analysis of associated

phenotypes. In: "Transmissible Subacute Spongiform Encephalopathies:

Prion Diseases". (Proceedings of the IIIrd International Symposium

on Transmissible Subacute Spongiform Encephalopathies: Prion

Diseases, 18-20 March 1996, Val de Grâce, Paris, France). L. Court

and B. Dodet, editors, Elsevier, Amsterdam, 1996, pp.433-444.

247. Brown, P. The risk of blood-borne Creutzfeldt-Jakob disease.

In: "Transmissible Subacute Spongiform Encephalopathies: Prion

Diseases". (Proceedings of the IIIrd International Symposium on

Transmissible Subacute Spongiform Encephalopathies: Prion Diseases,

18-20 March 1996, Val de Grâce, Paris, France). L. Court and

B. Dodet, editors, Elsevier, Amsterdam, 1996, pp.447-431.

Also, with slight modification:

Brown, P. The risk of blood-borne Creutzfeldt-Jakob disease.

In: Proceedings of the 4th NATO Military and Civil Blood Conference, The

Hague, The Netherlands, 5-8 May, 1996. Netherlands Military Medical

Review, vol.49 (June-December), pp.37-38.

248. Hainfellner, J.A., Liberski, P.P., Guiroy, D.C., Cervenáková, L.,

Brown, P., Gajdusek D.C. and Budka, H. Pathology and
immunohistochemistry of a kuru brain. Brain Pathology, 7: 1 (January),

547-554, 1997.

249. Epstein, L.G. and Brown, P. Bovine spongiform encephalopathy and a

new variant of Creutzfeldt-Jakob disease. Neurology, 48: 3 (March),

569-571, 1997.

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1997 (Con’t.)

250. Parchi, P. Capellari, S., Chen, S.G., Petersen, R.B., Gambetti, P.,

Kopp, N., Brown, P., Kitamoto T., Tateishi, J., Giese, A. and

Kretzschmar, H. Typing prion isoforms. Nature, 386: 6622

(March 20), 232-234, 1997.

251. Liberski, P.P., Brown, P., Cervenáková, L. and Gajdusek, D.C.

Interactions between astrocytes and oligodendroglia in human and

experimental Creutzfeldt-Jakob disease and scrapie. Experimental

Neurology, 144: 1 (March), 227-234, 1997.

252. El Hachimi, K.H., Chaunu, M.-P., Cervenáková, L., Brown, P. and

Foncin, J.-F. Putative neurosurgical transmission of Creutzfeldt-

Jakob disease with analysis of donor and recipient agent strains.

Comptes Rendus de l’Académie des Sciences (Paris), 320: 4 (April),

319-328, 1997.

253. Cervenáková, L., Rohwer, R., Williams, E.S., Brown, P. and

Gajdusek, D.C. High sequence homology of the PrP gene in mule deer

and Rocky Mountain elk. Lancet, 350: 9072 (July 19), 219-220, 1997.

254. McLean, C.A., Storey, E., Gardner, R.J.M., Tannenberg, A.E.G,

Cervenáková, L. and Brown, P. The D178N (cis-129M) "fatal familial

insomnia" mutation associated with diverse clinicopathological

phenotypes in an Australian kindred. Neurology, 49: 2 (August),

552-558, 1997.

255. Chen, S.G., Parchi, P., Brown, P., Capellari, S., Zou, W.,

Cochran, E.J., Vnencak-Jones, C.L., Julien, J., Vital, C., Mikol, J.,

Lugaresi, E., Autilio-Gambetti, L. and Gambetti, P. Allelic origin

of the abnormal prion protein isoform in familial prion diseases.

Nature Medicine, 3: 9 (September), 1009-1015, 1997.

256. Heckmann, J.G., Lang, C.J.G., Petruch, F., Druschky, A., Erb, C.,

Brown, P. and Neundörfer, B. Transmission of Creutzfeldt-Jakob

disease via a corneal transplant. Journal of Neurology,

Neurosurgery, and Psychiatry, 63: 3 (September), 388-390, 1997.

257. Brown, P. The risk of bovine spongiform encephalopathy ("mad cow

disease") to human health. Journal of the American Medical

Association, 278: 12 (September 24), 1008-1011, 1997.

258. Liberski, P.P., Brown, P. and Gajdusek, D.C. The evolution of views

on the nosological position of transmissible spongiform
encephalopathies. Folia Neuropathologica, 35: 4 (October-December),
214-225,

1997.

259. Walis, A., Liberski, P.P., Brown, P. and Gajdusek, D.C. Electron

microscopic studies of the optic nerve in experimental scrapie and

the panencephalopathic type of Creutzfeldt-Jakobd disease. Folia

Neuropathologica, 35: 4 (October-December), 255-258, 1997.

260. Martínez-Lage, J.F, Poza, M., Brown, P., Cervenáková, L., Bremón, A.R.

and de Pedro, J. Enfermedad de Creutzfeldt-Jakob en Neurocirugía:

una revisión de riesgos y medidas de prevención. Neurocirugía,

8: 4 (December), 284-293, 1997.

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1997 (Con’t.)

261. Brown, P. Spongiform encephalopathies: B-lymphocytes and

neuroinvasion. (News and Views). Nature, 390: 6661 (December

18/25), 662-663, 1997.

262. Brown, P., Cervenáková, L., McShane, L., Goldfarb, L.G., Bishop, K.,

Bastian, F., Kirkpatrick, J., Piccardo, P., Ghetti, B. and

Gajdusek, D.C. Creutzfeldt-Jakob disease in a husband and wife.

Neurology, 50: 3 (March), 684-688, 1998.

263. Brown, P. Donor pool size and the risk of blood-borne Creutzfeldt-

Jakob disease. Transfusion, 38: 3 (March), 312-315, 1998.

264. Zeidler, M. and Brown, P. More patients should be excluded from

being tissue donors. British Medical Journal, 316: 7138 (April 11),

1170-1171, 1998.

265. Brown, P. Commentary. BSE: the final resting place. Lancet, 351:

9110 (April 18), 1146-1147, 1998.

266. Fournier, J-G., Escaig-Haye, F., Billette de Villemeur, T., Robain,
O.,

Lasmézas, C.L., Deslys, J-P., Dormont, D. and Brown, P. Distribution

and submicroscopic immunogold localization of cellular prion protein

(PrPc) in extracerebral tissues. Cell and Tissue Research, 292: 1

(April), 77-84, 1998.

267. Heldt, N., Boellaard, J.W., Brown, P., Cervenáková, L.,

Doerr-Schott, J., Thomas, C., Scherer, C. and Rohmer, F.

Gerstmann-Sträussler-Scheinker disease with A117V mutation in

a second French-Alsatian family. Clinical Neuropathology, 17: 4

(July-August), 229-234, 1998.

268. Parchi, P., Chen, S.G., Brown, P., Zou, W., Capellari, S., Budka, H.,

Hainfellner, J., Reyes, P.F., Golden, G., Hauw, J.J., Gajdusek, D.C.

and Gambetti, P. Different patterns of truncated prion protein

fragments correlate with distinct phenotypes in P102L Gerstmann-

Sträussler-Scheinker disease. Proceedings of the National Academy

of Sciences (USA), 95: 14 (July 7), 8322-8327, 1998.

269. Brown, P. Commentary. On the origins of BSE. Lancet, 352: 9124

(July 25), 252-253, 1998.

270. Zanusso, G., Liu, D., Ferrari, S., Hegyi, I., Yin, X., Aguzzi, A.,

Hornemann, S., Liemann, S., Glockshuber, R., Manson, J.C., Brown, P.,

Petersen, R.B., Gambetti, P. and Sy, M-S. Prion protein expression

in different species: analysis with a panel of new mABs. Proceedings

of the National Academy of Sciences (USA), 95: 15 (July 21), 88128816,
1998.

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1998 (Con’t.)

271. McLean, C.A., Ironside, J.W., Alpers, M., Cervenáková, L.,

Anderson, R.McD., Brown, P.W. and Masters, C.L. Comparative

neuropathology of kuru with the new variant of Creutzfeldt-Jakob

disease: evidence for strain of agent predominating over genotype

of host. Brain Pathology, 8: 3 (July), 429-437, 1998.

272. Brown, P., Cervenáková, L. and Powers, J.M. FFI cases from the United

States, Australia, and Japan. Brain Pathology, 8: 3 (July), 567-570,

1998.

273. Chapman, J., Cervenáková, L., Petersen, R.B., Estupinan, J.,

Richardson, S., Vnencak-Jones, C.L., Gambetti, P., Gajdusek, D.C.,

Korczyn, A.D., Brown, P. and Goldfarb, L.G. APOE in non-Alzheimer

amyloidoses: transmissible spongiform encephalopathies. Neurology,

51, 2 (August), 548-553, 1998.

274. Brown P., Rohwer, R.G., Dunstan, B.C., MacAuley, C., Gajdusek, D.C.
and

Drohan, W.N. The distribution of infectivity in blood components and

plasma derivatives in experimental models of transmissible spongiform

encephalopathy. Transfusion, 38: 9 (September), 810-816, 1998.

275. Brown, P. Author’s Reply to a Letter to the Editor: Origins of BSE.

Lancet, 352: 9133 (September 26), 1068-1069, 1998.

276. Brown, P. Transmission of spongiform encephalopathy through biological

products. In: “Developments in Biological Standardization”.

Brown, F., Griffiths, E., Horaud, F. and Petricciani, J.C., editors.

Developments in Biological Standardization, volume 93, Karger, Basel,

1998, pp.73-78.

277. Brown, P., Cervenáková, L., McShane, L., Kleihues, P., Foncin, J.-F.,

Collins, G., Bastian, F., Goldfarb, L.G. and Gajdusek, D.C.

Polymorphic genotype matching in acquired Creutzfeldt-Jakob disease:

an analysis of donor/recipient case pairs. In “Prions and Brain

Diseases in Animals and Humans”. D.R.O. Morrison, editor. Nato ASI

Series A: Life Sciences volume 295, Plenum, New York, 1998, pp.19-24.

278. Piccardo, P., Dlouhy, S.R., Lievens, P.M.J., Young, K., Bird, T.D.,

Nochlin, D., Dickson, D.W., Vinters, H.V., Zimmerman, T.R.,

Mackenzie, I.R.A., Kish, S.J., Ang, L-C., De Carli, C.,

Pocchiari, M., Brown, P., Gibbs, C.J., Jr., Gajdusek, D.C.,

Bugiani, O., Ironside, J., Tagliavini, F. and Ghetti, B.

Phenotypic variability of Gerstmann-Sträussler-Scheinker disease

is associated with prion protein heterogeneity. Journal of
Neuropathology and Experimental Neurology, 57: 10 (October) 979-988, 1998.

279. Liberski, P.P., Barcikowska, M., Cervenáková, L., Bratosiewicz, J.,

Marczewska, M., Brown, P. and Gajdusek, D.C. A case of sporadic

Creutzfeldt-Jakob disease with a Gerstmann-Sträussler-Scheinker

phenotype but no alterations in the PRNP gene.

(Berlin), 96: 4 (October), 425-430, 1998.

Acta Neuropathologica

280. Liberski, P.P., Kordek, R., Brown, P. and Gajdusek, D.C. Astrocytes in

transmissible spongiform encephalopathies (prion diseases). Chapter 7

in: “Astrocytes in Brain Aging and Neurodegeneration”, Schipper,H.M.,

editor, R.G. Landes Co., Austin, Texas, 1998, pp.127-163.

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1998 (Con’t.)

281. Cervenáková, L., Goldfarb, L.G., Garruto, R., Lee, H.-E., Gajdusek,

D.C. and Brown, P. Phenotype-genotype studies in kuru: implications

for new variant Creutzfeldt-Jakob disease. Proceedings of the

National Academy of Sciences (USA), 95: 22 (October 27), 1323913241, 1998.

282. El Hachimi, K.H., Chaunu, M.-P., Brown, P. and Foncin, J.-F.

Modification of oligodendroglial cells in spongiform

encephalopathies. Experimental Neurology, 154: 1 (November),

23-30, 1998.

283. Brown, P. Transmissible spongiform encephalopathy. Chapter 43 in:

"Textbook of Clinical Neurology", Goetz, C.G. and Pappert, E.J.,

editors, W.B. Saunders Co., Philadelphia, 1998, pp.869-875.

284. Brown, P. Iatrogenic Creutzfeldt-Jakob disease. In: “Sterilization of

Medical Products”, Morrissey, R.F. and Kowalski, J.B., editors.

Proceedings of the VIIth International Kilmer Memorial Conference on

the Sterization of Medical Products, Scottsdale, Arizona, March 2-4,

1998. Polyscience Publications, Inc., Champlain NY, 1998, pp.212-218.

285. Brown, P. and Bradley, R. 1755 and all that: a historical primer of

transmissible spongiform encephalopathy. British Medical Journal,

317: 7174 (December 19-26), 1688-1692, 1998.

286. Hogan, R.N., Brown, P., Heck, E. and Cavanagh, H.D. Risk of prion

disease transmission from ocular donor tissue transplantation.

Cornea, 18: 1 (January/February), 2-11, 1999.

287. Bons, N., Mestre-Frances, N., Belli, P., Cathala, F., Gajdusek, D.C.

and Brown, P. Natural and experimental oral infection of nonhuman

primates by bovine spongiform encephalopathy agents. Proceedings of

the National Academy of Sciences (USA), 96: 7 (March 30); 4046-4051,

1999.

288. Lee, H.S., Sambuughin, N., Cervenáková, L., Chapman, J., Pocchiari,
M.,

Litvak, S., Qi, H.Y., Budka, H., del Ser, T., Furukawa, H.,

Brown, P., Gajdusek, D.C., Long, J.C., Korczyn, A. and Goldfarb, L.G.

Ancestral origins and worldwide distribution of the PRNP 200K

mutation causing familial Creutzfeldt-Jakob disease. American

Journal of Human Genetics, 64: 4 (April), 1063-1070, 1999.

289. Grigoriev, V., Escaig-Haye, F., Streichenberger, N., Kopp, N.,

Langeveld, J., Brown, P. and Fournier, J-G. Submicroscopic
immunodetection of PrP in the brain of a patient with a new-variant of

Creutzfeldt-Jakob disease. Neuroscience Letters, 264: 1-3 (April 2),

57-60, 1999.

290. Brown, P., Bradley, R. and Cathala, F. Bref historique des
encéphalopathies spongiformes transmissibles. Revue du Praticien: 49: 9

(May 1), 928-933, 1999.

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1999 (Con’t.)

291. Parchi, P., Giese, A., Capellari, S., Brown, P., Schulz-Schaeffer, W.,

Windl, O., Zerr, I., Budka, H., Kopp, N., Piccardo, P., Poser, S.,

Rojiani, A., Streichemberger, N., Julien, J., Vital, C., Ghetti, B.,

Gambetti, P. and Kretzschmar, H. Classification of sporadic

Creutzfeldt-Jakob disease based on molecular and phenotypic analysis

of 300 subjects. Annals of Neurology, 46, 2 (August), 224-233, 1999.

292. Brandel, J-P., Cathala, F. and Brown, P. Maladies à prions:

encéphalopathies spongiformes subaiguës transmissibles humaines et

animales. Chapitre 4 in: Neurogénétique. Affections hérédodégénératives,
A. Brice and D.F. Schorderet, editors. Doin, Paris,

1998, pp.35-61.

293. Brown, P. A brief history of scrapie before the prion. In:”The Science

and Culture Series”, K. Goebel, editor. International Seminar on

Nuclear War and Planetary Emergencies (23rd Session), Erice, Italy,

19-24 August 1998. World Scientific, Singapore, 1999, pp.35-36.

294. Brown, P. Transmissible spongiform encephalopathies. Chapter 59 in:

“Neurology in Clinical Practice”, W.G. Bradley, R.B. Daroff, G.M.

Fenichel and C.D. Marsden, editors. Butterworth-Heinemann, Newton

MA, 1999, volume II, pp.1423-1430.

295. Parchi, P., Brown, P., Capellari, S., Gibbs, C.J., Jr. and
Gambetti, P.

Agent strain variation in human prion diseases: insight from

transmission to primates. In: “Alzheimer’s Disease and Related

Disorders”, K. Iqbal, D.R. Swaab, B. Winblad and H.M. Wisniewski,

editors. John Wiley & Sons, London, 1999, pp.561-567.

Also as abstract:

Parchi, P., Brown, P., Capellari, S., Gibbs, C.J., Jr. and Gambetti, P.

Biochemical analysis of strain variation in human prion diseases:

insight from transmission to primates. Abstract No.722 in Book of

Abstracts, 6th International Conference on Alzheimer’s Disease and

Related Disorders, Amsterdam, July 18-23, 1998

296. Brown, P. The risk of blood-borne Creutzfeldt-Jakob disease. In:

“Developments in Biologicals”, Brown, F. and Vyas, G., editors.

Karger, Basel, 1999, volume 102, pp.53-59.

297. Boellaard, J.W., Brown, P. and Tateishi J. Gerstmann-Sträussler-

Scheinker disease -the dilemma of molecular and clinical

correlations. Clinical Neuropathology, 18, 6 (November-December),

271-285, 1999.

298. Brown, P., Cervenáková, L., McShane, L.M., Barber, P., Rubenstein, R.

and Drohan, W.N. Further studies of blood infectivity in an

experimental model of transmissible spongiform encephalopathy, with

an explanation of why blood products do not transmit Creutzfeldt-

Jakob disease. Transfusion, 39: 11/12 (November-December),

1169-1178, 1999.

299. Cervenáková, L., Buetefisch, C., Lee, H-S., Taller, I., Stone, G.,

Gibbs, C.J., Jr., Brown, P., Hallett, M. and Goldfarb, L.G. Novel

PRNP sequence variant associated with familial encephalopathy.

American Journal of Medical Genetics (Neuropsychiatric Genetics),

88: 6 (December), 653-656, 1999.

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1999 (Con’t.)

300. Dickson, D.W. and Brown, P. Multiple prion types in the same brain.

Is a molecular diagnosis of CJD possible? Neurology, 53: 9

(December), 1903-1904, 1999.

2000

301. Blanquet-Grossard, F., Sazdovitch, V., Jean, A., Deslys, J-P.,

Dormont, D., Hauw, J-J., Marion, D., Brown, P. and Cesbron, J.-Y.

Prion protein is not detectable in dental pulp from patients with

Creutzfeldt-Jakob disease. Journal of Dental Research, 79: 2

(February), 700, 2000.

302. Brown, P., Rau, E.H., Johnson, B.K., Bacote, A.E., Gibbs, C.J., Jr.
and

Gajdusek D.C. New studies on the heat resistance of hamster-adapted

scrapie agent: threshold survival after ashing at 600°C suggests an

inorganic template of replication. Proceedings of the National

Academy of Science (USA), 97: 7 (March 28), 3418-3421, 2000.

303. Brown, P. and Lamb, G. Creutzfeldt-Jakob disease and the mortuary

profession. The Director, 72: 3 (March), 58-62, 2000.

304. Majtényi, C., Brown, P., Cervenáková, L., Goldfarb, L.G. and
Tateishi, J.

A three-sister sibship of Gerstmann-Sträussler-Scheinker disease with

a CJD phenotype. Neurology, 54: 6 (June), 2133-2137, 2000.

305. Brown, P. and Cervenáková, L. Authors’ Reply to a Letter to the

Editor: Infectivity of buffy coat in variant CJD. Transfusion, 40: 6

(June), 754-755.

306. Fournier, J.C., Kopp, N., Streichenberger, N., Escaig-Haye, F.,

Langeveld, J. and Brown, P. Electron microscopy of brain amyloid

plaques from a patient with new variant Creutzfeldt-Jakob disease.

Acta Neuropathologica, 99: 6 (June), 637-642, 2000.

307. Parchi, P., Zou, W., Wang, W., Brown, P., Capellari, S., Ghetti, B.,

Kopp, N., Schulz-Schaeffer, W.J., Kretzschmar, H.A., Head, M.W.,

Ironside, J.W., Gambetti, P. and Chen, S.G. Genetic influence on the

structural variations of the abnormal prion protein. Proceedings of

the National Academy of Science (USA), 97: 18 (August 29), 1016810172,
2000.

308. Liberski, P.P., Bratosiewicz, J., Barcikowska, M., Cervenakova, L.,

Marczewska, M., Brown, P. and Gajdusek, D.C. A case of sporadic

Creutzfeldt-Jakob disease with a Gerstmann-Sträussler-Scheinker

phenotype but no alterations in the PRNP gene.

100: 2 (August), 233-234, 2000.

Acta Neuropathologica

309. Cervenáková, L., Protas, I.I., Hirano, A., Votiakov, V.I.,

Nedzved, M.K., Kolomiets, N.D., Taller, I., Park, K.-Y.,

Sambuughin, D., Gajdusek, D.C., Brown, P. and Goldfarb, L.G.

Progressive muscular atrophy variant of familial amyotrophic

lateral sclerosis (PMA/ALS). Journal of the Neurological Sciences,

177: 2 (August), 124-130, 2000.

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2000 (Con’t.)

310. Brown, P. BSE and transmission through blood [Commentary].

356: 9233 (September 16), 955-956, 2000.

Lancet,

311. Brown, P., Preece, M., Brandel, J.-P., Sato, T., McShane, L.,

Zerr, I., Fletcher, A., Will, R.G., Pocchiari, M., Cashman, N.R.,

d’Aignaux, J.H., Cervenáková, L., Fradkin, J., Schonberger, L.B. and

Collins, S.J. Iatrogenic Creutzfeldt-Jakob disease at the Millennium.

Neurology, 55: 8 (October 24), 1075-1081, 2000.

312. Goldfarb, L.G., Bütefisch, C. and Brown, P. Ataxia in the
transmissible

spongiform encephalopathies. In: Handbook of Ataxia Disorders,

T. Klockgether, editor. Marcel Dekker, New York, 2000, pp.523-543.

313. Brown, P. Risk of Creutzfeldt-Jakob disease associated with blood or

blood products. Proceedings of an INFARMED Workshop held in Evora,

Portugal, December 11-12, 1999. In: Jornada Sobre Medicamentos:

Hemoderivados e vCJD, J.A. da Silva, editor. INFARMED, Lisboa, 2000,

pp.79-84.

314. WHO Infection Control Guidelines for Transmissible Spongiform

Encephalopathies. Report of a WHO Consultation, Geneva, Switzerland,

23-26 March 1999. WHO/CDS/CSR/APH/2000.3

2001

315. Brown, P., Cervenáková, L. and Diringer, H. Blood infectivity and

the prospects for a diagnostic screening test in Creutzfeldt-Jakob

disease. Journal of Laboratory and Clinical Medicine, 137: 1

(January), 5-13, 2001. [Erratum 137: 4 (April), 230, 2001]

316. Lee, H.-S., Brown, P., Cervenáková, L., Garruto, R.M., Alpers, M.P.,

Gajdusek, D.C. and Goldfarb, L.G. Increased susceptibility to kuru

of PRNP 129 MM genotype carriers. Journal of Infectious Diseases,

183: 1 (January); 192-196, 2001.

317. Brown, P., Will, R.G., Bradley, R., Asher, D.M. and Detwiler, L.

Bovine spongiform encephalopathy and variant Creutzfeldt-Jakob

disease: background, evolution, and current concerns. Emerging

Infectious Diseases, 7: 1(January/February), 6-16, 2001.

318. Brown, P. The pathogenesis of transmissible spongiform encephalopathy:

routes to the brain and the possibility of therapeutic barricades.

Cellular and Molecular Life Sciences, 58: 1 (February): 259-265,

2001.

319. Brown, P. Authors’ Reply to a Letter to the Editor: Iatrogenic

Creutzfeldt-Jakob disease at the millennium. Neurology, 56: 7

(April 10) 987, 2001.

320. Liberski, P.P., Bratosiewicz, J., Walis, A., Kordek, R., Jeffrey, M.

and Brown, P. A special report. I.Prion protein (PrP) – amyloid

plaques in the transmissible spongiform encephalopathies, or prion

diseases revisited. Folia Neuropathology, 39: 4 (April), 217-235,

2001.

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2001 (Con’t.)

321. Kennedy, R.H., Hogan, R.N., Brown, P., Holland, E., Johnson, R.T.,

Stark, W. and Sugar, J. Eye banking and screening for Creutzfeldt-

Jakob disease. Archives of Ophthalmology, 119: 5 (May); 721-726,

2001.

322. Brown, P. Afterthoughts about bovine spongiform encephalopathy and

variant Creutzfeldt-Jakob disease. Emerging Infectious Diseases,

7: 3 (June Supplement), 598-600, 2001.

323. Brown, P.

tests.

2001.

Creutzfeldt-Jakob disease: blood infectivity and screening

Seminars in Hematology, 38: 4 (October, Supplement 9), 2-6,

324. Brown, P. Transmissible Spongiform Encephalopathies. Chapter 29 in

“Baker’s Clinical Neurology”. R.J. Joynt and R.C. Griggs, editors.

CD-ROM, Lippincott Williams & Wilkins, Baltimore, 2001.

325. Panegyres, P.K., Toufexis, K., Kakulas, B.A., Cervenáková, L.,

Brown, P., Ghetti, B., Piccardo, P. and Dlouhy, S.R. A new PRNP

mutation (G131V) associated with Gerstmann-Sträussler-Scheinker

disease. Archives of Neurology, 58: 11 (November), 1899-1902, 2001.

326. Brown, P. Transmissible spongiform encephalopathies. In: “Early Onset

Dementia”, Hodges, J.R., editor. Oxford University Press, Oxford,

2001, pp.367-384.

327. Brown P. The scientist’s dilemma. In: “Proceedings of Joint

WHO/FAO/OIE Technical consultation on BSE: public health, animal

health and trade”, Paris, 11-14 June, 2001, pp.74-75.

328. Bons, N., Lehmann, S., Nishida, N., Mestre-Frances, N., Dormont, D.,

Belli, P., Delacourte, A., Grassi, J. and Brown, P. BSE infection of

the small short-lived primate Microcebus murinus. Comptes Rendus de

l’Academie de Science (Paris) 325: 12 (December), 1-8, 2001.

329. Cervenáková, L., Brown, P., Hammond, D.J., Lee, C.A. and Saenko, E.L.

Factor VIII and transmissible spongiform encephalopathy: the case for

safety. Hemophilia, 8: 2 (March) 63-75, 2002.

330. Brown P. What are the current human risks from mad cow disease? MD

Consult Infectious Disease, May 9, 2002. http://www.mdconsult.com

331. Liberski, P.P., Bratosiewicz-Wasik, J., Gajdusek, D.C. and Brown, P.

Ultrastructural studies of experimental scrapie and Creutzfeldt-Jakob

disease in hamsters. I. Alterations of myelinated axons. Acta

Neurobiologiae Experimentalis, 63: 3 (June), 121-129, 2002.

332. Liberski, P.P., Bratosiewicz-Wasik, J., Gajdusek, D.C. and Brown, P.

Ultrastructural studies of experimental scrapie and Creutzfeldt-Jakob

disease in hamsters. II. Astrocytic and macrophage reaction towards

axonal destruction. Acta Neurogiologiae Experimentalis, 63: 3

(June), 131-139, 2002.

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2002 (Con’t.)

333. Liberski, P.P., Gajdusek, D.C. and Brown, P. How do neurons degenerate

in prion diseases or transmissible spongiform encephalopathies

(TSE’s): neuronal autophagy revisited. Acta Neurogiologiae

Experimentalis, 63: 3 (June), 141-147, 2002.

334. Brown, P. Current issues related to the origin and etiology of BSE.

Relationship with variant Creutzfeldt-Jakob disease. In: “Report

of the PAHO/WHO Consultation on Bovine Spongiform Encephalopathy:

Scientific Bases for Policy Decisions in the Americas”, Montevideo,

Uruguay, 9-11 April 2001. Veterinary Public Health Program, Pan

American Health Organization, Washington, D.C., 2002.

335. Bons, N., Lehmann, S., Mestre-Francès, N., Dormont, D. and Brown, P.

Brain and buffy coat transmission of bovine spongiform encephalopathy

(BSE) to the primate Microcebus murinus. Transfusion, 42: 5 (May),

513-517, 2002.

336. Brown, P. Drug therapy in human and experimental transmissible

spongiform encephalopathy. Neurology, 58: 12 (June 25), 1720-1725,

2002.

337. Brown, P. Transmission of Creutzfeldt-Jakob disease by transfusion.

In: “Rossi’s Principles of Transfusion Medicine”, 3rd Edition.

T.L. Simon, W.H. Dzik, E.L. Snyder, C.P. Stowell and R.G. Strauss,

editors. Lippincott Williams & Wilkins, Philadelphia, 2001,

pp.784-788.

338. Dagvadorj, A., Petersen, R.B, Lee, H.S., Cervenakova,L, Budka, H.,

Boyle, R., Brown, P., Gambetti, P. and Goldfarb, L.G. Spontaneous

mutations in the prion protein gene causing transmissible spongiform

encephalopathy. Annals of Neurology, 52: 9 (September), 355-359,

2002.

339. Stenland C.J., Lee, D.C., Brown, P., Ironside, J., Petteway, S.R., Jr.

and Rubenstein, R. Partitioning of human and sheep forms of the

pathogenic prion protein during the purification of therapeutic

proteins from human plasma. Transfusion, 42: 11 (November),

1497-1500, 2002.

340. Brown, P. Special precautions for autopsies of patients with

Creutzfeldt-Jakob disease. Chapter 13 in: “Autopsy Performance and

Reporting”, K.A. Collins and G.M. Hutchins, editors, College of

Amercian Pathologists, Northfield, Illinois, 2003, pp.105-110.

341. Cervenáková, L., Brown, P., Soukharev, S., Yakovleva, O., Diringer,
H.,

Saenko, E.L. and Drohan, W.N. Failure of immunocompetitive

capillary electrophoresis assay to detect disease-specific prion

protein in buffy coat from humans and chimpanzees with Creutzfeldt-

Jakob disease. Electrophoresis, 24: 6 (March), 853-859, 2003.

20


Case 1:06-cv-00544-JR Document 14-9 Filed 11/03/2006 Page 32 of 47

2003 (Con’t.)

342. Ricketts, M. and Brown, P. Transmissible spongiform encephalopathy:

update and implications for blood safety. Clinics in Laboratory

Medicine, 23: 1 (March), 129-137, 2003.

343. Liberski, P.P., Sikorska, B., Bratosiewicz-Wasik, J., Walis, A,

Brown, P. and Brown, D. Exuberant cellular reaction of the optic

nerves in experimental Creutzfeldt-Jakob disease. Acta

Neurobiologiae Experimentalis, 63: 3 (March), 309-318, 2003.

344. Brown, P., Transmissible Spongiform Encephalopathy as a Zoonotic

Disease. ILSI Europe Report Series, International Life Sciences

Institute, ILSI Press, Brussels, March 2003, 47 pp.

345. Brown, P. Variant CJD transmission through blood: risks to predictors

and “predictees”. Transfusion, 43: 4 (April), 425-427, 2003.

346. Brown, P., Meyer, R., Cardone, F. and Pocchiari, M.
Ultra-highpressure inactivation of prion infectivity in processed meat: a

practical method to prevent human infection. Proceedings of the

National Academy of Science (USA), 100: 10 (May 13), 6093-6097,

2003.

347. WHO Guidelines on Transmissible Spongiform Encephalopathies in
relation

to Biological and Pharmaceutical Products. Report of a WHO

Consultation, Geneva, Switzerland, 3-5 February 2003.

http://www.who.int/bloodproducts/tse/en

348. Brown, P. Creutzfeldt-Jakob disease (CJD). In: “Encyclopedia of the
Neurological Sciences”,

M.J. Aminoff and R.B. Daroff, editors, Academic Press, San Diego, 2003,
volume 1, pp.795802.

349. Brown, P. Transmissible Spongiform Encephalopathy. Chapter 43 in:

“Textbook of Clinical Neurology”, 2nd Edition, C.G. Goetz, editor,

Saunders, Philadelphia, 2003, pp.945-954.

350. Brandel, J.-P., Preece, M., Brown, P., Croes, E., Laplanche J.-L.,

Will, R. and Alpérovitch, A. Distribution of codon 129 genotypes

in human growth hormone-treated CJD patients in France and the

United Kingdom. Lancet, 362: 9378 (July 12), 128-130, 2003.

351. Walis, A., Bratosiewicz, J., Sikorska, B., Brown, P., Gajdusek, D.C

and Liberski, P.P. Ultrastructural changes in the optic nerves of

rodents with experimental Creutzfeldt-Jakob disease (CJD),

Gerstmann-Sträussler-Scheinker disease (GSS) or scrapie. Journal of

Comparative Pathology, 129: 2-3: (August-October), 213-225, 2003.

352. Cervenakova, L., Yakovleva, O., McKenzie, C., Kolchinsky, S.,

McShane, L., Drohan, W.N. and Brown, P. Similar levels of

infectivity in the blood of mice infected with human-derived vCJD

and GSS strains of transmissible spongiform encephalopathy.

Transfusion, 43: 12 (December), 1687-1694, 2003.

21


Case 1:06-cv-00544-JR Document 14-9 Filed 11/03/2006 Page 33 of 47

353. Brown, P. Fall-out from a possible transfusion-related transmission of

vCJD. The Lancet Neurology, 3: 4 (April), 203, 2004.

354. Mills, J.L., Schonberger, L.B., Wysowski, D.K., Brown, P., Durako,

S.J., Cox, C., Kong, F. and Fradkin, J.E. Long Term Mortality in

the United States cohort of pituitary-derived growth hormone

recipients. J Pediatrics, 2004 144: 4 (April), 430-436, 2004.

355. Sikorska, B., Walis A., Bratosiewicz-Wasik, J., Brown, P. and

Liberski, P.P. Fate of myelinated fibres in the optic nerves in

experimental Creutzfeldt-Jakoc disease in rodents: an

ultrastructural study. Folia Neuropathologica 42: 2 (April-June),

101-105, 2004.

356. Liberski, P.P. and Brown, P. Kuru: a half-opened window onto the

landscape of neurodegenerative diseases. Folia Neuropathologica 42:

Supplement A (June), 3-14, 2004.

357. Liberski, P.P. and Brown, P. Prion diseases: from transmission

experiments to structural biology – still searching for the cause.

Folia Neuropathologica, 42: Supplement A (June), 15-32, 2004.

358. Brown, P. Mad cow disease in cattle and human beings. American

Scientist, 92: 4 (July-August), 334-341, 2004.

359. Fichet, G., Comoy, E., Duval, C., Antloga, K., Dehen, C.,
Charbonnier, A.,

McDonnell, G., Brown, P., Lasmézas, C.I. and Deslys, J-P. Novel

methods for disinfection of prion-contaminated medical devices.

Lancet, 364: 9433 (August 7), 521-526, 2004.

360. Brown, P. and Cervenáková, L., The modern landscape of
transfusion-related

iatrogenic Creutzfeldt-Jakob disease and blood screening tests. Current

Opinion in Hematology, 11: 5 (September), 351-356, 2004.

361. Brown, P., Rau, E.H., Meyer, R., Lemieux, P., Cardone, F. and

Pocchiari, M. ‘Extreme’ inactivation methods for transmissible

spongiform encephalopathy agents. In: “Proceedings of the Eighth

International Kilmer Conference”, J.B. Kowalski and R.F. Morrissey,

editors, Osaka, Japan, October 6-9, 2003. Polyscience Publications

Inc., Laval, Canada, 2004, pp.104-111.

362. Brown, P., Rau, E.H., Lemieux, P., Johnson, B.K., Bacote, A. and

Gajdusek, D.C. Infectivity studies of both ash and air emissions

from simulated incineration of scrapie-contaminated tissues.

Environmental Science and Technology, 38: 22 (November 15),

6155-6160, 2004.

363. Yakovleva, O., Janiak, A., McKenzie, C., McShane, L., Brown, P. and

Cervenakova, L. Effect of protease treatment on plasma infectivity

in variant Creutzfeldt-Jakob disease mice. Transfusion, 44: 12

(December), 1700-1705, 2004.

364. Cervenáková, L. and Brown, P. Advances in test development for

transmissible spongiform encephalopahies. Expert Review of

Anti-infective Therapy, 2: 6 (December), 873-880, 2004.

22


Case 1:06-cv-00544-JR Document 14-9 Filed 11/03/2006 Page 34 of 47

2005

365. Brown, P. and Abee, C.R. Working with transmissible spongiform

encephalopathy agents. Institute for Laboratory Animal Research

(ILAR) Journal, 46: 1 (January), 44-52, 2005.

366. Brown, P. and Cervenáková L. A prion lexicon (out of control).

Lancet, 365: 9454 (January 8), 122, 2005.

367. Lasmézas, C., Comoy, E., Hawkins, S., Herzog, C., Mouthon, F.,

Konold, T., Auvré, F., Correia, E., Lecoutra-Etchegaray, N.,

Salès, N., Wells, G., Brown, P. and Deslys, J.P. Risk of oral

infection with bovine spongiform encephalopathy agent in primates.

Lancet, 365: 9461 (February 26), 781-783, 2005.

368. Brown, P. Pathogenesis and transfusion risk of transmissible

spongiform encephalopathies. In: “Advances in Transfusion Safety”,

G. Vyas and A. Williams, editors, Developments in Biologicals,

volume 120, Karger, Basel, 2005, pp.27-33.

369. Brown, P. Blood infectivity, processing and screening tests in

transmissible spongiform encephalopathies. Vox Sanguinis,

89: 2 (August), 63-70, 2005.

370. Sowemimo-Coker, S., Kascsak, R., Kim, A., Andrade, F., Pesci, S.,

Kascsak, R., Meeker, C., Carp, R. and Brown, P. Removal of

exogenous (spiked) and endogenous prion infectivity from red cells

with a new prototype of leukocyte reduction filter. Transfusion,

45: 12 (December), 1839-1844, 2005.

371. Kennedy, R.H., Mills, C.R. and Brown, P. Risk of infectious disease

transmission through the use of Allografts. In: “Essentials in

Ophthalmology: Oculoplastics and Orbit”, R. Guthoff and

J. Katowitz, editors, Springer-Verlag, Berlin, 2005, pp.3-18.

2006

372. Thomzig, A., Cardone, F., Krüger, D., Pocchiari, M., Brown, P. and

Beekes, M. Pathological prion protein in muscles of hamsters and

mice infected with rodent-adapted BSE or vCJD. Journal of General

Virology, 87: 1 (January), 252-254, 2006.

373. Cardone, F., Brown, P., Meyer, R., and Pocchiari, M. Inactivation of

transmissible spongiform encephalopathy agents in food products by

ultra-high pressure-temperature treatment. Biochimica et Biophysica

Acta (Proteins and Proteomics), 1764: 3 (March), 558-562, 2006.

374. Walis, A, Liberski P.P. and Brown, P. Ultrastructural alterations

in the optic nerve in transmissible spongiform encephalopathies – a

review. Folia Neuropathologica 42: Supplement B (March), 153-160, 2006.

375. Liberski, P.P., Jaskolski, M. and Brown, P. Gerstmann-Sträussler-

Scheinker disease. II. An effect of GSS mutation on PRP structure.

Folia Neuropathologica 42: Supplement B (March), 153-160, 2006.

23


Case 1:06-cv-00544-JR Document 14-9 Filed 11/03/2006 Page 35 of 47

2006 (Con’t)

376. Liberski, P.P. and Brown, P. Astrocytes in transmissible spongiform

encephalopathies (prion diseases). Folia Neuropathologica 42:

Supplement B (March), 153-160, 2006.

377. Brown, P. The quest for a pre-clinical blood screening test for TSE.

Neuroprion News, No. 5 (April), 2006, p.1.

378. Brown, P. Letter to God. British Medical Journal. 332: 7553 (3 June),

1341.

379. Brown, P. Blood infectivity in the transmissible spongiform

encephalopathies. Chapter 4 in: “Creutzfeldt-Jakob Disease:

Managing the Risk of Transmission by Blood, Plasma, and Tissues”,

Turner M.L., editor. ABBA Press, Bethesda, MD, 2006, pp. 95-118.

380. Minor, P. and Brown P. Diagnostic tests for CJD for ante-mortem

screening. Chapter 5 in: “Creutzfeldt-Jakob Disease: Managing the

Risk of Transmission by Blood, Plasma, and Tissues”, Turner M.L.,

editor. ABBA Press, Bethesda, MD, 2006, pp. 119-148.

381. Baribeau, A-M., Bradley, R.B., Brown, P., Goodwin, J., Kihm, U.,

Lotero, E., O’Conner D., Schuppers, M., and Taylor D. Biodiesel

from Specified Risk Material Tallow: an Appraaisal of TSE Risks and

Their Reduction. ATF Advanced Technologies & Fuels Canada, Inc.,

Ottowa, July 2006.

382. Brown, P., Brandel, J.-P., Preece, M. and Sato, T. Iatrogenic

Creutzfeldt-Jakob disease: the waning of an era. Neurology,

67:3 (August 8), 389-393.

^^^. Brown, P. Atypical BSE. Neuroprion News, No. 6 (November), 2006, p.1.

^^^. Brown, P., McShane, L. Zanusso G and Detwiler, L. On the question of

sporadic or atypical bovine spongiform encephalopathy and

Creutzfeldt-Jakob disease. Emerging Infectious Diseases, 2006

[IN PRESS]

^^^. Brown, P. and Detwiler, L. Bovine spongiform encephalopathy. In: Food

Microbiology: Fundamentals and Froniers”, 3rd edition, Doyle, M.F,

^^^^^^^^^^^^^^^^^^^^^^, editors. ASM Press, Washington D.C.,

pp.^^^-^^^. [IN PRESS]

^^^. Sikorska, B., Liberski, P. and Brown, P. Neuronal autophagy and

aggresomes constitute a significant part of neurodegenerations in

experimental scrapie. [IN PRESS]

2007

^^^. Georgsson, G., Sigurdarson, S. and Brown, P. The infectious agent of

sheep scrapie may survive in the environment for at least sixteen

years. Journal of General Virology, [IN PRESS]

24


Case 1:06-cv-00544-JR Document 14-9 Filed 11/03/2006 Page 36 of 47

2007 (Con’t)

^^^. Williams, L., Brown, P., Ironside, J., Gibson, S., Will, Rl, Ritchie,

D., Kreil, T.R.and Abee, C. Clinical, neuropathological, and

immunohistochemical features of sporadic and variant forms of

Creutzfeldt-Jakob disease in the squirrel monkey (Saimiri sciureus}.

Journal of General Virology, 2007. [IN PRESS]

^^^. Larramendy-Gozalol, C., Berret, A, Daudigeos E, Mathieu, E.,

Antonangeli, L., Riffet, C., Petit, E., Papy-Garcia, D.,

Barritault, D., Brown, P. and Deslys, J.-P. Characterization of

CR36, a new heparan mimetic, and Pentosan Polysulfate in the

treatment of prion diseases.

[SUBMITTED]

Journal of General Virology, 2007

^^^. Brown, P. Transmissible spongiform encephalopathy. Chapter 43 in:

“Textbook of Clinical Neurology”, 3rd edition, Goetz, C., editor.

W.B. Saunders, Philadelphia, 2007, pp. ^^^-^^^. [IN PRESS]

^^^. Martinez-Lage, J.F., Brown, P. and Martin, P.M. Enfermedades por

priones adquiridas: enfermedad de Creutzfeld-Jakob yatrógena.

Chapter 11 in: “^^^^^^^^^^^^^^^^^^^^^^ [IN PRESS]

^^^. Liberski, P.P., Brown, D.R., Caughey, B. and Brown, P. Neuronal cell

death caused by a misfolded protein in transmissible spongiform

encephalopathies (prion diseases).

^^^-^^^, 2006 [IN PRESS].

Autophagy 2: 3 (Oct/Nov/Dec),

^^^. Pan, T., Sethi, J., Nelsen, C., Cervenakova, L., Lohman, K.,

Wegrzyn, R., Rucolph, A., Brown, P. and Orser, C.S. Evidence for

PRPTSE in blood by the misfolded protein diagnostic Assay.

Transfusion, [IN PREPARATION]

25


Case 1:06-cv-00544-JR Document 14-9 Filed 11/03/2006 Page 37 of 47

ATTACHMENT B

Paul W. Brown, M.D.

Curriculum Vitae


Case 1:06-cv-00544-JR Document 14-9 Filed 11/03/2006 Page 38 of 47

CURRICULUM VITAE

Present address

Birthdate/Place

Education and

experience

Society

affiliations

Paul W. Brown, M.D.

7815 Exeter Road

Bethesda, Maryland 20814

Telephone (301) 652-5940

Fax (301) 652-4312

e-mail paulwbrown@comcast.net

12 March 1936, Hackensack, New Jersey, USA

A.B. (Magna cum Laude), Harvard College, 1957

M.D., The Johns Hopkins School of Medicine, 1961

Internship and 1st year medical residency:

Osler Medical Service, The Johns Hopkins Hospital,

1961-1963

Research Associate, NINDS, NIH, 1963-1965.

Staff Associate, National Institute of Child Health and

Human Development, (NICHHD), NIH, 1965-70

2nd year medical residency, University of California San

Francisco Medical Center, 1965-66

3rd year medical residency, Osler Medical Service,

The Johns Hopkins Hospital, 1966-67

Chargé de Recherche, L'Institut National de la Santé et de

la Recherche Médicale (INSERM), and Medical

Consultant, American Embassy, Paris, 1971-72

Staff Associate, NINDS, NIH, 1971-89

Visiting Scientist, INSERM, Laboratoire de

Neurovirologie, Clinique des Maladies du Système

Nerveux, Hôpital de la Salpêtrière, Paris, 1977-78

Medical Director, US Public Health Service, 1979-1999

Senior Investigator, NINDS, 1990-2004

American College of Physicians

American Epidemiological Society

Infectious Diseases Society of America

American Society for Virology

Société Française de Neurologie

American Neurological Association


Case 1:06-cv-00544-JR Document 14-9 Filed 11/03/2006 Page 39 of 47

Languages English and French

Publications Over 380 papers during a span of 40 years, mainly dealing with

transmissible spongiform encephalopathy (TSE), and especially the topics

of epidemiology, infectivity, and inactivation

Editorial European Journal of Epidemiology, Associate editor,
1990positions Journal of the Neurological Sciences, Associate editor,
1991-97

Honors and -Henry Strong Denison Scholar, The Johns Hopkins School

recognitions School of Medicine, 1961

-Alpha Omega Alpha (AOA), Johns Hopkins Chapter, 1961

-Diplomate, American Board of Internal Medicine, 1968

-Prix Léopold Trasbot, Académie Vétérinaire de France,

1980

-Fellow, National Multiple Sclerosis Society, 1971-72

-Fellow, Committee to Combat Huntington’s Chorea, 1973

-Chairman, DHHS Interagency Epidemiology

Subcommittee on Human Growth Hormone and

Creutzfeldt-Jakob disease, 1985 to present

-Lawson Wilkins Pediatric Endocrine Society Lecture,

Los Angeles, California, 1987

-Thomas Campione Lecture,

Northwestern University School of Medicine, 1987

-USPHS Commendation Medal, 1990

-Arnold Barnett Lecture,

Wichita Society of Neuroscience, 1991

-Member, WHO Expert Advisory Panel on Neurosciences,

1991-1993

-Consultant to PAHO (Pan American Health Organization)

for the evaluation of bovine spongiform encephalopathy

in Latin America, 1992

-USPHS Outstanding Service Medal, 1992

-Consultant to EEC Biomed 1/2 Project: Surveillance of

Creutzfeldt-Jakob disease in the European Community,

1992 to present

-Andrew Mark Lippard Memorial Lecture, College of Physicans

and Surgeons of Columbia University, 1995

-Chairman, WHO consultation on TSE and Medical Products

(1997)

-Transmissible Spongiform Encephalopathies Advisory

Committee, Center for Biologics Evaluation and Research,

FDA: Chairman 1997-2001; Ad hoc member 2001-present

-Chairman, Neurodegererative Diseases Working Group,

World Federation of Scientists, 1998-2000

2


Case 1:06-cv-00544-JR Document 14-9 Filed 11/03/2006 Page 40 of 47

Honors and -Chairman, WHO consultation on TSE and Infection Control

recognitions Guidelines (1999)

(continued) -Distinguished Scientist Seminar Lecturer,

University of South Alabama School of Medicine, 2000

-Marie C. and Joseph C. Wilson Memorial Lecture,

University of Rochester Medical Center, 2000

-Bill Stone Distinguished Speaker, 2001

South Texas Blood & Tissue Center

-Convocation Lecture and Seminar, Berea College, 2001

-Fredrich Deinhardt Lectureship, 18th Annual Clinical Virology

Symposium, Clearwater Beach, Florida, 2002

-Board of Governors and Board of Scientific Directors,

The Memorial Institute for Neurodegenerative Diseases

of Saskatchewan, Canada, 2003

-Eagleson Lecture, American Biological Safety Association

Conference, 2004

-Plenary Lecture, European Network of Excellence Neuroprion

Conference, Paris, 2004

-Co-Chairman, Dominique Dormont Memorial Conference,

Paris, 2005

-Co-President, Fondation Alliance de Biotechnologie,

Paris, 2006 onwards

3


Case 1:06-cv-00544-JR Document 14-9 Filed 11/03/2006 Page 41 of 47

ATTACHMENT C

Brown, P, McShane, LM, Zanusso, G, Detwiler, L, On the question of
sporadic or atypical

bovine spongiform encephalopathy (BSE) and Creutzfeldt-Jakob disease


Case 1:06-cv-00544-JR Document 14-9 Filed 11/03/2006 Page 42 of 47

PERSPECTIVE

On the Question of Sporadic

or Atypical Bovine SpongiformEncephalopathy and

Creutzfeldt-Jakob Disease

Paul Brown,* Lisa M. McShane,† Gianluigi Zanusso,‡ and Linda Detwiler§

Strategies to investigate the possible existence of sporadic

bovine spongiform encephalopathy (BSE) require

systematic testing programs to identify cases in countries

considered to have little or no risk for orally acquired disease,

or to detect a stable occurrence of atypical cases in

countries in which orally acquired disease is disappearing.

To achieve 95% statistical confidence that the prevalence

of sporadic BSE is no greater than 1 per million (i.e., the

annual incidence of sporadic Creutzfeldt-Jakob disease

[CJD] in humans) would require negative tests in 3 million

randomly selected older cattle. A link between BSE and

sporadic CJD has been suggested on the basis of laboratory

studies but is unsupported by epidemiologic observation.

Such a link might yet be established by the discovery

of a specific molecular marker or of particular combinations

of trends over time of typical and atypical BSE and various

subtypes of sporadic CJD, as their numbers are influenced

by a continuation of current public health measures that

exclude high-risk bovine tissues from the animal and

human food chains.

Bovine spongiform encephalopathy (BSE) was first recognized

in 1986 in the United Kingdom and quickly

reached epidemic proportions, affecting >30,000 cattle per

year by 1992. Because of continuing exportation of both

live cattle and meat and bone meal rendered from the carcasses

of slaughtered cattle, the disease spread throughout

most of Europe and a few non-European countries.By

2006, 20 years after its first appearance in the United

Kingdom, the disease had been reported in an additional

24 countries (1).

*Bethesda, Maryland, USA; †National Institutes of Health,

Bethesda, Maryland, USA; ‡University of Verona, Verona, Italy;

and §Virginia-Maryland Regional College of Veterinary Medicine,

College Park, Maryland, USA

Beginning toward the end of the 1980s in the United

Kingdom, and in the 1990s in other countries, numerous

regulations were enacted to minimize the entry of contaminated

tissues into both the animal and human food chains

and to eliminate the international spread of disease. These

measures have been extraordinarily successful, to the

extent that no new countries have been added to the list

during the past year and the number of new cases has dramatically

diminished in most countries in which BSE has

appeared (the situation in some countries with insufficient

surveillance remains unclear).

Although the origin of the epidemic is thought to have

been caused by a species-crossing contamination by sheep

scrapie during the course of rendering and recycling carcass

meat and bone meal as cattle feed, an alternative

hypothesis suggested an origin in a similarly recycled case

of spontaneously occurring disease in cattle. The pros and

cons of these competing ideas have been argued elsewhere

(2,3), and neither will ever be convincingly proved or disproved.

Thus, the phenomenon of spontaneous disease

remained in limbo until the recent discovery of “atypical”

strains of BSE reopened the question. In this article we

consider the importance of atypical BSE within the overall

concept of sporadic (spontaneous) disease and whether

such cases, if they exist, could account for at least some

cases of apparently sporadic Creutzfeldt-Jakob (CJD) in

humans.

Sporadic BSE

Obviously, the ideal country in which to examine the

question of sporadic BSE would have a large national herd

that was guaranteed never to have been exposed to environmental

sources of infection. Such an ideal will never be

realized. Until recently, the United States appeared to have

at least approached the ideal by having a large national

Emerging Infectious Diseases • www.cdc.gov/eid • Vol. 12, No. 12,
December 2006


Case 1:06-cv-00544-JR Document 14-9 Filed 11/03/2006 Page 43 of 47

herd, an adequate testing program, and an apparently small

risk for contamination by imported cattle or cattle feed.

That position was made vulnerable in late 2003 by the discovery

of a case of BSE imported from Canada and was

eliminated altogether by the subsequent discovery of 2

indigenously infected animals in widely separate regions

of the country. Although the 2 indigenous cases might represent

sporadic disease, the continuing identification of

cases in western Canada, coupled with a history of substantial

numbers of cattle imported from Canada into the

United States (both indigenous US animals had the same

molecular “signature” as the most recent Canadian case),

makes it difficult to ignore the possibility of undetected

instances of feed contamination from imported and recycled

infectious carcasses.

At present, the 2 best countries in which to undertake

testing programs would be Argentina and Australia; both

have large national herds (˜50 million and 30 million animals,

respectively), and both are considered to be free of

orally acquired BSE infections, on the basis of importation

history, nutritional practices, and adequacy of surveillance

(4). Even in these countries, however, the discovery of a

case of BSE could not be guaranteed to be spontaneous

because of the widespread global distribution of potentially

infected cattle and cattle feed and the vagaries of international

trade: imperfect record keeping, lack of

compliance, and just plain deception.

By way of illustration, an incident occurred many years

ago that involved a particularly bulky shipment labeled as

a pesticide. The large quantity seemed unusual to the customs

inspector, who opened it and discovered that the shipment

contained meat and bone meal destined to be spread

on fields to inhibit grazing by deer, a serious agricultural

pest. Thus, a study of sporadic BSE would only be truly

convincing if no cases were identified.

Moreover, the criteria for answering the question of

sporadic BSE are different than for orally acquired BSE.

Most importantly, we do not know at what age sporadic

cases of BSE might occur, but they are unlikely to be in the

3- to 5-year-old age group in which orally acquired BSE is

most prevalent. If the age distribution of sporadic disease

in cattle were to mimic that of sporadic CJD in humans, it

would not peak until 14–20 years of age (the last third of

the ˜20-year natural life span of a cow). Substantial numbers

of such older cattle do not exist, and thus it may never

be possible to state with assurance that spontaneous BSE

does not occur.

Even if we accept this practical constraint, we can still

take advantage of the fact that in many countries a proportion

of the total slaughter population consists of breeding

stock and dairy cows that are culled at >7 years of age, and

animals that go directly to rendering plants or die “on

farm” further increase this number. Argentina, for exam

Bovine Spongiform Encephalopathy and CJD

ple, with a national herd of ˜50 million cattle, in 2005

recorded nearly 1.4 million deaths from slaughter and natural

causes in animals >7 years (L. Mascitelli, pers.

comm.).

Approximately 10% of cases of sporadic CJD occur in

patients 25–50 years of age; this age in humans corresponds

to the middle third of a cow’s normal life span, or

7–13 years of age (Figure 1). If the age distribution of sporadic

BSE followed the same pattern, negative test results

in a total of ˜3 million animals randomly selected from

this group would allow us to be 95% confident that sporadic

BSE is not present at a prevalence >1 per million,

and ˜4.5 million negative animals would raise the level of

confidence to 99%. Larger numbers of BSE-negative animals

would be required to achieve these levels of confidence

for a maximum prevalence <1 per 10 million cattle

(Table 1, Figure 2).

Even the least rigorous negative result—a prevalence

not greater than that of sporadic CJD in humans, or 1 per

million—would require several years to achieve, and it is

perhaps unrealistic to suppose that the motivation to prolong

the testing program will endure much beyond the

global disappearance of orally acquired BSE and variant

CJD. Nevertheless, to the degree that testing older as well

as younger adult animals approached these numbers, both

statistical and consumer confidence would increase, and at

the very least provide reassurance that the occurrence of

sporadic disease must be exceedingly rare, with little likelihood

of posing a risk to either human or animal nutrition.

Atypical BSE

Because of its contemporary nature, the study of atypical

BSE is very much a work in progress, with comparatively

little published data and many unknowns. The first 2

cases to be identified were a serendipitous discovery made

in the course of an unrelated experimental study that

required a detailed neuropathologic and immunochemical

Figure 2. Maximum prevalence according to number of negative

cattle at 95% (solid line) and 99% (dashed line) confidence levels.

See Table 1 for exact numbers and statistical method.

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Case 1:06-cv-00544-JR Document 14-9 Filed 11/03/2006 Page 44 of 47

PERSPECTIVE

examination of the entire brain (5). The absence of clinical

signs in these older animals, the unusual distribution of

PrPTSE, together with amyloid plaques, and a Western blot

pattern that differed from the stereotypic pattern seen in

typical BSE left little doubt about the probability that a

new “atypical strain” had been identified (bovine amyloidotic

spongiform encephalopathy[BASE]).

Although no further cases were found in nearly 200 cattle

examined in Italy, the initiation of Western blot studies

of animals in other countries with screening test programs

began to yield additional atypical patterns (Table 2, Figure

3) (6–14; P. Lind, pers. comm.). Two major patterns have

been described, named L (resembling the original Italian

case pattern with a lower molecular weight than typical

BSE) and H (for a distinct pattern first seen in France with

a higher molecular weight than typical BSE). It is not yet

clear whether other mixed patterns result from technical

procedures in different laboratories or whether a more

complicated scheme of classification will evolve as more

atypical patterns are discovered.

In addition, Western blots of PrPTSE are a fragile basis

on which to define a BSE phenotype. Little or no information

is available about either the clinical status or neuropathologic

features of these animals. We know that cases

have occurred in different breeds and PrP genotypes, and

we also know that very few of the animals have had the

typical clinical picture of BSE (behavioral disturbances,

sensory signs, ataxia, and tremors), but a cloud of ambiguity

surrounds the clinical picture even in those animals for

which an extensive post-hoc investigation was undertaken.

The fact that few detailed neuropathologic results are

available is explained by the need to preserve at least a full

half brain for examination, which is presently not done in

any of the various countries that have screening test programs.

In the future, the brain as well as the carcass must

be retained in cold storage until the test results are known.

The frequency of atypical cases is another unknown.

Published (7,12) and unpublished (11,13) observations

indicate that in some countries it might be as high as

5%–10% of the total number of older animals diagnosed

by rapid screening tests (e.g., 2/27 in Germany, and 1/9 in

Canada), which would seem to be a surprisingly high proportion

of spontaneously occurring cases. However, data

are not yet sufficient to estimate the overall prevalence of

atypical BSE, i.e., cases per million tested animals of all

ages.

In this context, a word is in order about the US testing

program. After the discovery of the first (imported) cow in

2003, the magnitude of testing was much increased, reaching

a level of >400,000 tests in 2005 (Figure 4). Neither of

the 2 more recently indigenously infected older animals,

with ambiguous or no clinical features, would have been

detected without such testing, and neither would have been

identified as atypical without confirmatory Western blots.

Despite these facts, surveillance has now been decimated

to 40,000 annual tests (USDA news release no. 0255.06,

July 20, 2006) and invites the accusation that the United

Figure 1. Distribution of ages at onset of illness in 500 cases of

neuropathologically verified or experimentally transmitted sporadic

Creutzfeldt-Jakob disease. Approximately 10% of cases occur in

patients during the middle third (25–49 years) of a human lifespan,

which corresponds to age in cattle of ~7–13 years.

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Case 1:06-cv-00544-JR Document 14-9 Filed 11/03/2006 Page 45 of 47

Bovine Spongiform Encephalopathy and CJD

States will never know the true status of its involvement

with BSE.

In short, a great deal of further work will need to be

done before the phenotypic features and prevalence of

atypical BSE are understood. More than a single strain

may have been present from the beginning of the epidemic,

but this possibility has been overlooked by virtue of the

absence of widespread Western blot confirmatory testing

of positive screening test results. These new phenotypes

may be found, at least in part, to result from infections at

an older age by a typical BSE agent, rather than neonatal

infections with new “strains” of BSE. Neither alternative

has yet been investigated.

Sporadic CJD

The possibility that at least some cases of apparently

sporadic CJD might be due to infection by sporadic cases

of BSE cannot be dismissed outright. Screening programs

needed to identify sporadic BSE have yet to be implemented,

and we know from already extant testing programs that

at least a proportion of infected animals have no symptoms

and thus would never be identified in the absence of systematic

testing. Thus, sporadic BSE (or for that matter,

sporadic disease in any mammalian species) might be

occurring on a regular basis at perhaps the same annual

frequency as sporadic CJD in humans, that is, in the range

of 1 case per million animals.

Whether humans might be more susceptible to atypical

forms of BSE cannot be answered at this time.

Experimentally transmitted BASE shows shorter incuba

tion periods than BSE in at least 1 breed of cattle,

bovinized transgenic mice, and Cynomolgus monkeys

(12,13). In humanized transgenic mice, BASE transmitted,

whereas typical BSE did not transmit (13). Paradoxically,

the other major phenotype (H) showed an unusually long

incubation period in bovinized transgenic mice (12).

The limited experimental evidence bearing on a possible

relationship between BSE and sporadic CJD is difficult

to interpret. The original atypical BASE strain of BSE had

a molecular protein signature very similar to that of 1 subtype

(type 2 M/V) of sporadic CJD in humans (5).

In another study, a strain of typical BSE injected into

humanized mice encoding valine at codon 129 showed a

Figure 3. Representation of Western blots of PrPTSE patterns of

typical bovine spongiform encephalopathy (BSE) and the 2 major

types of atypical BSE. M.W., molecular weight in kilodaltons; L

type, atypical “light” pattern; H type, atypical “heavy” pattern.

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December 2006


Case 1:06-cv-00544-JR Document 14-9 Filed 11/03/2006 Page 46 of 47

PERSPECTIVE

Figure 4. Numbers of tested cattle in the United States,

2000–2007. Number tested in 2006 as of October; number tested

in 2007 proposed by the US Department of Agriculture.

glycopattern indistinguishable from the same subtype of

sporadic CJD (15). In a third study, the glycopatterns of

both the H and L strains of atypical BSE evidently did not

resemble any of the known sporadic CJD subtypes (12).

To these molecular biology observations can be added

the epidemiologic data accumulated during the past 30

years. The hypothesis that at least some cases of apparently

sporadic CJD are due to unrecognized BSE infections

cannot be formally refuted, but if correct, we might expect

by now to have some epidemiologic evidence linking BSE

to at least 1 cluster of apparently sporadic cases of CJD.

Although only a few clusters have been found (and still

fewer published), every proposed cluster that has been

investigated has failed to show any common exposure to

bovines. For that matter, no common exposure has been

shown to any environmental vehicles of infection, including

the consumption of foodstuffs from bovine, ovine, and

porcine sources, the 3 livestock species known to be susceptible

to transmissible spongiform encephalopathies.

Additional negative evidence comes from several large

case-control studies in which no statistically significant

dietary differences were observed between patients with

sporadic CJD and controls (16,17).

On the other hand, the difficulty of establishing a link

between BSE and CJD may be compounded by our ignorance

of the infectious parameters of a sporadic form of

BSE (e.g., host range, tissue distribution of infectivity,

route of transmission, minimum infectious dose for

humans, whether single or multiple). Presumably, these

parameters would resemble those of variant CJD' that is,

high infectivity central nervous system and lymphoreticular

tissues of an infected cow find their way into products

consumed by humans. Transmissions that might have

occurred in the past would be difficult to detect because

meat products are generally not distributed in a way that

results in detectable geographic clusters.

Barring the discovery of a specific molecular signature

(as in variant CJD), the most convincing clue to an association

will come from the observation of trends over time of

the incidence of typical and atypical BSE and of sporadic

and variant CJD. With 4 diseases, each of which could

have increasing, unchanging, or decreasing trends, there

could be 81 (34) possible different combinations. However,

it is highly likely that the trends for typical BSE and variant

CJD will both decrease in parallel as feed bans continue

to interrupt recycled contamination. The remaining

combinations are thus reduced to 9 (32), and some of them

could be highly informative.

For example, if the incidence of atypical BSE declines

in parallel with that of typical BSE, its candidacy as a sporadic

form of disease would be eliminated (because sporadic

disease would not be influenced by current measures

to prevent oral infection). If, on the other hand, atypical

BSE continues to occur as typical BSE disappears, this

would be a strong indication that it is indeed sporadic, and

if in addition at least 1 form of what is presently considered

as sporadic CJD (such as the type 2 M/V subtype

shown to have a Western blot signature like BASE) were

to increase, this would suggest (although not prove) a

causal relationship (Figure 5).

Recognition of the different forms of BSE and CJD

depends upon continuing systematic testing for both

bovines and humans, but bovine testing will be vulnerable

Figure 5. Diagram of 2 possible informative trends in the incidence

of bovine spongiform encephalopathy (BSE) and Creutzfeld-Jakob

disease (CJD). The left panel shows the likely trends of typical

BSE and variant CJD (vCJD). The right upper panel shows 1 possible

pair of trends of atypical BSE and sporadic CJD (sCJD)

that might occur in conjunction with the typical BSE/vCJD trends,

and would be consistent with the interpretation that atypical BSE

is not sporadic and not related to sCJD. The right lower panel

shows a second possible associated pair of trends consistent with

the interpretation that atypical BSE is sporadic and might also be

related to the type 2 M/V subset of apparently sCJD.

Emerging Infectious Diseases • www.cdc.gov/eid • Vol. 12, No. 12,
December 2006


Case 1:06-cv-00544-JR Document 14-9 Filed 11/03/2006 Page 47 of 47

to heavy pressure from industry to dismantle the program

as the commercial impact of declining BSE cases ceases to

be an issue. Industry should be aware, however, of the

implications of sporadic BSE. Its occurrence would necessitate

the indefinite retention of all of the public health

measures that exclude high-risk bovine tissues from the

animal and human food chains, whereas its nonoccurrence

would permit tissues that are now destroyed to be used as

before, once orally acquired BSE has disappeared.

Acknowledgments

We thank Victoria E. Bridges and Chris Kopral for providing

data about annual cattle slaughter numbers from the Food

Safety and Inspection Service of the US Department of

Agriculture (USDA) and for estimates of cattle dying on farms

from data supplied by the National Animal Health Monitoring

System, Animal and Plant Inspection Services, Veterinary

Service, USDA.

This study was funded in part by grant # 4AN/F10 “Studio

dei meccanismi patogenetici delle malattie neurodegenerative per

la diagnosi e lo sviluppo di approcci terapeutici” from the Istituto

Superiore di Sanità, Rome, Italy

Dr Brown has recently retired after a 41-year career in the

Laboratory of CNS Studies at the National Institutes of Health,

where he focused on studying transmissible spongiform

encephalopathies.

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Address for correspondence: Paul Brown, 7815 Exeter Rd, Bethesda, MD

20814, USA; email: paulwbrown@comcast.net

Emerging Infectious Diseases • www.cdc.gov/eid • Vol. 12, No. 12,
December 2006
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