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From: TSS ()
Subject: Isolated visual symptoms at onset in sporadic Creutzfeldt-Jakob disease: the clinical phenotype of the "Heidenhain variant"
Date: September 16, 2005 at 5:53 pm PST

British Journal of Ophthalmology 2005;89:1341-1342; doi:10.1136/bjo.2005.074856
© 2005 by BMJ Publishing Group Ltd


EXTENDED REPORT

Isolated visual symptoms at onset in sporadic Creutzfeldt-Jakob disease: the clinical phenotype of the "Heidenhain variant"
S A Cooper, K L Murray, C A Heath, R G Will and R S G Knight
The National Creutzfeldt-Jakob Disease Surveillance Unit, Western General Hospital, Crewe Road, Edinburgh EH4 2XU, UK


Correspondence to:
Dr Sarah Cooper
The National Creutzfeldt-Jakob Disease Surveillance Unit, Western General Hospital, Crewe Road, Edinburgh EH4 2XU, UK; sarah.cooper@doctors.org.uk


Background: The Heidenhain variant of sporadic Creutzfeldt-Jakob disease (sCJD) is commonly understood to represent cases with early, prominent visual complaints. The term is clarified to represent those who present with isolated visual symptoms. This group may pose diagnostic difficulties and often present to ophthalmologists where they may undergo needless invasive procedures.

Method: A retrospective review of 594 pathologically proved sCJD cases referred to the UK National CJD Surveillance Unit over a 15 year period to identify Heidenhain cases.

Results: 22 cases had isolated visual symptoms at onset with a mean illness duration of 4 months. The mean age at disease onset was 67 years. Most displayed myoclonus, pyramidal signs, and a delay in the onset of dementia for some weeks. 17 (77%) were referred initially to ophthalmology. Two underwent cataract extraction before diagnosis. All tested cases were homozygous for methionine at codon 129 of the prion protein gene.

Conclusions: This rare, but clinically distinct, group of patients with sCJD may cause diagnostic difficulties. Because ocular intervention carries with it the risk of onward transmission awareness of this condition among ophthalmologists is important.

--------------------------------------------------------------------------------

Abbreviations: EEG, electroencephalogram; MRI, magnetic resonance imaging; NCJDSU, National CJD Surveillance Unit; sCJD, sporadic Creutzfeldt-Jakob disease


Keywords: Creutzfeldt-Jakob disease; visual loss; Heidenhain variant

http://bjo.bmjjournals.com/cgi/content/abstract/89/10/1341?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=prion&searchid=1126914357767_1861&stored_search=&FIRSTINDEX=0&volume=89&issue=10&journalcode=bjophthalmol

Greetings,

>>>Because ocular intervention carries with it the risk of onward transmission awareness of this condition among ophthalmologists is important. <<<

i had the same concerns and posted many years ago. but with no PhDs, my warnings were meaningless.

they finally come around and get off the damn nvCJD only theory. it's about time...tss

tss

-------- Original Message --------
Subject: Eye procedure raises CJD concerns
Date: Fri, 19 Nov 2004 08:19:35 -0600
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
To: BSE-L@UNI-KARLSRUHE.DE

Eye procedure raises CJD concerns


By Steve Mitchell
Medical Correspondent

Washington, DC, Nov. 18 (UPI) -- A New York man who died from a rare
brain disorder similar to mad cow disease in May underwent an eye
procedure prior to his death that raises concerns about the possibility
of transmitting the fatal disease to others, United Press International
has learned.

The development comes on the heels of the announcement Thursday by U.S.
Department of Agriculture officials of a possible second case of mad cow
disease in U.S. herds.

Richard Da Silva, 58, of Orange County, N.Y., died from Creutzfeldt
Jakob disease, an incurable brain-wasting illness that strikes about one
person per million.

Richard's wife Ann Marie Da Silva told UPI he underwent a check for the
eye disease glaucoma in 2003, approximately a year before his death. The
procedure involves the use of a tonometer, which contacts the cornea --
an eye tissue that can contain prions, the infectious agent thought to
cause CJD.

Ann Marie's concern is that others who had the tonometer used on them
could have gotten infected.

A 2003 study by British researchers suggests her concerns may be
justified. A team led by J.W. Ironside from the National
Creutzfeldt-Jakob Disease Surveillance Unit at the University of
Edinburgh examined tonometer heads and found they can retain cornea
tissue that could infect other people -- even after cleaning and
decontaminating the instrument.

"Retained corneal epithelial cells, following the standard
decontamination routine of tonometer prisms, may represent potential
prion infectivity," the researchers wrote in the British Journal of
Ophthalmology last year. "Once the infectious agent is on the cornea, it
could theoretically infect the brain."

Prions, misfolded proteins thought to be the cause of mad cow, CJD and
similar diseases, are notoriously difficult to destroy and are capable
of withstanding most sterilization procedures.

Laura Manuelidis, an expert on these diseases and section chief of
surgery in the neuropathology department at Yale University, agreed with
the British researchers that tonometers represent a potential risk of
passing CJD to other people.

Manuelidis told UPI she has been voicing her concern about the risks of
corneas since 1977 when her own study, published in the New England
Journal of Medicine, showed the eye tissue, if infected, could transmit CJD.

At the time the procedure was done on Richard Da Silva, about a year
before he died, she said it was "absolutely" possible he was infectious.

The CJD Incidents Panel, a body of experts set up by the U.K. Department
of Health, noted in a 2001 report that procedures involving the cornea
are considered medium risk for transmitting CJD. The first two patients
who have a contaminated eye instrument used on them have the highest
risk of contracting the disease, the panel said.

In 1999, the U.K. Department of Health banned opticians from reusing
equipment that came in contact with patients' eyes out of concern it
could result in the transmission of variant CJD, the form of the disease
humans can contract from consuming infected beef products.

Richard Da Silva was associated with a cluster of five other cases of
CJD in southern New York that raised concerns about vCJD.

None of the cases have been determined to stem from mad cow disease, but
concerns about the cattle illness in the United States could increase in
light of the USDA announcement Thursday that a cow tested positive on
initial tests for the disease. If confirmed, this would be the second
U.S. case of the illness; the first was detected in a Washington cow
last December. The USDA said the suspect animal disclosed Thursday did
not enter the food chain. The USDA did not release further details about
the cow, but said results from further lab tests to confirm the initial
tests were expected within seven days.

Ann Marie Da Silva said she informed the New York Health Department and
later the eye doctor who performed the procedure about her husband's
illness and her concerns about the risk of transmitting CJD via the
tonometer.

The optometrist -- whom she declined to name because she did not want to
jeopardize his career -- "didn't even know what this disease was," she said.

"He said the health department never called him and I called them (the
health department) back and they didn't seem concerned about it," she
added. "I just kept getting angrier and angrier when I felt I was being
dismissed."

She said the state health department "seems to have an attitude of don't
ask, don't tell" about CJD.

"There's a stigma attached to it," she said. "Is it because they're so
afraid the public will panic? I don't know, but I don't think that the
answer is to push things under the rug."

New York State Department of Health spokeswoman Claire Pospisil told UPI
she would look into whether the agency was concerned about the
possibility of transmitting CJD via tonometers, but she had not called
back prior to story publication.

Disposable tonometers are readily available and could avoid the risk of
transmitting the disease, Ironside and colleagues noted in their study.
Ann Marie Da Silva said she asked the optometrist whether he used
disposable tonometers and "he said 'No, it's a reusable one.'"

Ironside's team also noted other ophthalmic instruments come into
contact with the cornea and could represent a source of infection as
they are either difficult to decontaminate or cannot withstand the harsh
procedures necessary to inactivate prions. These include corneal burrs,
diagnostic and therapeutic contact lenses and other coated lenses.

Terry Singletary, whose mother died from a type of CJD called Heidenhain
Variant, told UPI health officials were not doing enough to prevent
people from being infected by contaminated medical equipment.

"They've got to start taking this disease seriously and they simply
aren't doing it," said Singletary, who is a member of CJD Watch and CJD
Voice -- advocacy groups for CJD patients and their families.

U.S. Centers for Disease Control and Prevention spokeswoman Christine
Pearson did not return a phone call from UPI seeking comment. The
agency's Web site states the eye is one of three tissues, along with the
brain and spinal cord, that are considered to have "high infectivity."

The Web site said more than 250 people worldwide have contracted CJD
through contaminated surgical instruments and tissue transplants. This
includes as many as four who were infected by corneal grafts. The agency
noted no such cases have been reported since 1976, when sterilization
procedures were instituted in healthcare facilities.

Ironside and colleagues noted in their study, however, many disinfection
procedures used on optical instruments, such as tonometers, fail. They
wrote their finding of cornea tissue on tonometers indicates that "no
current cleaning and disinfection strategy is fully effective."

Singletary said CDC's assertion that no CJD cases from infected
equipment or tissues have been detected since 1976 is misleading.

"They have absolutely no idea" whether any cases have occurred in this
manner, he said, because CJD cases often aren't investigated and the
agency has not required physicians nationwide report all cases of CJD.

"There's no national surveillance unit for CJD in the United States;
people are dying who aren't autopsied, the CDC has no way of knowing"
whether people have been infected via infected equipment or tissues, he
said.

Ann Marie Da Silva said she has contacted several members of her state's
congressional delegation about her concerns, including Rep. Sue Kelly,
R-N.Y., and Sen. Charles Schumer, D-N.Y.

"Basically, what I want is to be a positive force in this, but I also
want more of a dialogue going on with the public and the health
department," she said.

Copyright 2004 United Press International

http://www.washtimes.com/upi-breaking/20041118-030642-2974r.htm

The Eyes have it/CJD * and they could be stealing them from YOUR loved
one, hence the
spread of CJD (aka MADCOW DISEASE) will spread...


http://jama.ama-assn.org/issues/v282n23/full/jlt1215-5.html

http://mad-cow.org/~tom/dec99_news.html#bbb

Testimony of Bess Believeaux, Lions Eye Bank of Central Texas
(Submission to the Jan. 18/19 meeting of the
TSE Advisory Committee)

http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_16.pdf

TSS Submission to the same Committee;
http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_09.pdf

Tissue Banks International (TBI), Gerald J Cole
http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_13.pdf

Mom's autopsy report
DIVISION OF NEUROPATHOLOGY
University of Texas Medical Branch
114 McCullough Bldg.
Galveston, Texas 77555-0785

FAX COVER SHEET

DATE: 4-23-98

TO: Mr. Terry Singeltary @ -------

FROM: Gerald Campbell

FAX: (409) 772-5315 PHONE: (409) 772-2881


Number of Pages (including cover sheet):

Message:


*CONFIDENTIALITY NOTICE*

This document accompanying this transmission contains
confidential information belonging to the sender that is legally
privileged. This information is intended only for the use of the
individual or entry names above. If you are not the intended recipient,
you are hereby notified that any disclosure, copying distribution, or
the taking of any action in reliances on the contents of this telefaxed
information is strictly prohibited. If you received this telefax in
error, please notify us by tlephone immediately to arrange for return
of the original documents.
--------------------------
Patient Account: 90000014-518
Med. Rec. No.: (0160)118511Q
Patient Name: POULTER, BARBARA Age: 63 YRS DOB: 10/17/34
Sex: F Admitting Race: C

Attending Dr.:
Date / Time Admitted : 12/14/97 1228
Copies to:

UTMB
University of Texas Medical Branch
Galveston, Texas 77555-0543
(409) 772-1238 Fax (409) 772-5683
Pathology Report

FINAL AUTOPSY DIAGNOSIS
Autopsy' Office (409)772-2858

Autopsy NO.: AU-97-00435

AUTOPSY INFORMATION:
Occupation: Unknown Birthplace: Unknown Residence: Crystal Beach
Date/Time of Death: 12/14/97 13:30 Date/Time of Autopsy: 12/15/97 15:00
Pathologist/Resident: Pencil/Fernandez Service: Private
Restriction: Brain only

FINAL AUTOPSY DIAGNOSIS

I. Brain: Creutzfeldt-Jakob disease, Heidenhain variant.

***TYPE: Anatomic(A) or Clinical(C) Diagnosis.
IMPORTANCE: 1-immediate cause of death (COD); 2.ureterlying COD;
3-contributory COD: 4.concomitant, significant; 5-incidental ***

Patient Name: POULTER, BARBARA
Patlenttc~'ation: AUTOPSY
Room/Bed:
Printed Date; Time: 01/30/98 - 0832

Page: 1
Continued ....
--------------

UTMB
University of Texas Medical Branch
Galveston, Texas 77555-0543
(409) 772-1238 Fax (409) 772-5683

Pathology Report

Autopsy NO,: AU-97-00435

MICROSCOPIC DESCRIPTION:
The spongiform change is evident in all areas of neocortex, varying
from mild to moderate in severity with only very mild neuronal loss and
gliosis. In the bilateral occipital lobes, there is severe loss
cortical neurons and gliosis, with a corresondinq pallor of the
underlying white matter. There is only minimal, focal spongiform change
in corpus striatum, lentiform nuclei, thalamus, hippocampus, brainstem
and cerebellum. There is no significant loss of neurons from the lateral
geniculate nucleus, and the optic chiasm and tracts are well-myelinated.

SECTIONS TAKEN:
N-l) Pituitary, N-2) Right frontal, N-3) Right inferior frontal, N-4)
Right caudate putamen. N-5) Right lentiform nuclei, N-6) Right
hippocampus, N-7) optic chiasm. N-8) Left inferior temporal lobe, N-9)
Right inferior occipital, N-10} Cerabellum. N-l1) Midbrain, N-12) Pons,
N-13) Medulla.

FINAL DIAGNOSES:
BRAIN:
1. Clinical history of rapidly progressive dementia, clinically
consistent with Creutzfeldt-Jakob Disease.

a. spongiform encephalopathy, most Severe in occipital lobes, consistent
with Heidenhain variant of Creutzfeldt-Jakob disease.

b. Ventriculer enlargement, moderate, consistent with atrophy.
1. Communicating spherical enlargement of occipital horn of left
lateral ventricle (possible incidental congenital anomaly.

DURA; Left subdural hemorrhage, recent, minimal.

PITUITARY: Severe capillary congestion.

COMMENTS; See also western blot report from Dr. Gambetti's lab
Amyloid stains are not completed for this case as of this date. The
results, which are not essential for the diagnosis, will be reported
separately in an addendum.

(this was hand written notes)
no anyloid evident in the special stains.
no evidence of paques.

Gerald A. Campbell, M.D., Pathologist
Division of Neuropathology

(Electronic Signature}.
(Gross: 01/16/98
Final: 02/08/98

Patient Name: POULTER, BARBARA
Patient Location: AUTOPSY
Room/Bed:
Printed Date: Time: 02/09/98 - 1120

Page 2
END OF REPORT
-------

UTMB
University of Texas Medical Branch
Galveston, Texas 77555-0543
(409) 772-1238
fax (409) 772-5683
Pathology Report


Date/Time of Death: 12/14/97
Autopsy No.: AU-97-00435

NEUROPATHOLOGY CONSULTATION

CLINICAL HISTORY
This patient was a 63-year-old white female with recent onset of
progressive dementia. She was well until September of this year, when
she noted a decrease in her visual activity and was found to have visual
field defets as well. MRI revealed no lesions in the orbits or optic
pathways. She was admitted to the hospital with the working diagnosis
of bilateral optic neuropathy for a course of intravenous
methylprednisolone, but her vision continued to deteriorate. She
developed increasing memory and speech impairment, weakness and
myoclonus. She died on 12/14/97, approximately three and one-half
months after her symptoms started.

Date/Time of Death: 12/14/97 13:30 Date/Time Autopsy: 12/15/97 15:00
Pathologist Resident: PENCIL/FERNANDEZ

GROSS DESCRIPTION:
Submitted are the brain, convexity dura and pituitary gland.

The pituitary gland is very dark and almost hemorrhagic in appearance,
but has no obvious hematoma. It is submitted totally for histology.

The right convexity dura has diffuse but minimal subdura hemorrhage,
and the dura is otherwize unremarkable.

The brain is normally developed with normal size for an adult and is
symmetric externally. It does not have apparent sulcal widening. There
is mild congestion of thc leptomeninges, which are transparent. There is
no evidence of inflammatory exudete. There is no evidence of internal
softenings or other lesions externally. The cerebral arteries have focal
atherosclerosis, but are without significant compromise of the vessels
lumens. There im no evidence of aneurysms or malformations.

The hemispheres are sliced coronally revealing, a ventricular system
which is mildly enlarged. The cortical ribbon is normal in thickness
throuqhout most of the brain, except for the inferior amd medial
occipital lobes bilaterally, where the cortex is firm, thin and has a
brownish discoloration, more severely so on the left than the right. In
addition there is a spherical enlargement of the left occipital horn of
the lateral ventricle which communicates with the remainder of the
lateral ventricle. Tho tissue of the white matter around this
enlargement is somewhat softer then in other areas. Other areas of the
brain are grossly unremarkable. The brainstem and cerebellum are sliced
transversely, revealing normal development and no evidence of gross
changes or lesions.

DICTATED BY: GERALD A. CAMPBELL, M.D., PATHOLOGIST
01/16/98

Page 1
Continued ....
---------------

Patient Account: 90000014-518
Med. Rec. No,: (0160)118511Q

Patient Name: POULTER, BARBARA
Age: 63 YRS DOB: 10/17/34 Sex:F Race:C
Admitting Dr.:
Attending Dr:
Date/Time Admitted: 12/14/97 1228

UTMB
University of Texas Medical Branch
Galveston, Texas 77555-0543
(409) 772-1238 Fax (409) 772-5683
Pathology Report

FINAL AUTOPSY REPORT
Autopsy Office (409)772-2858
Autopsy No.: AU-97-00435

CLINICAL SUMMARY:

This is a 63-year-old white female with a recent onset of progressive
dementia. Her past medical history is significant for hypothyroidism.
She was well until September of this year, when she noted visual
difficulty. By mid-October, she could not read the newspaper. She was
found to have a decrease in visual acuity and visual field defects. One
week after her initial evaluation, a panel of blood tests showed no
significant abnormalities and a MRI revealed some periventricular white
matter "plaque-like" areas but no lesions in the orbits or optic
pathways.

The patient had continued deterioration and distortion of her vision.
The visual field defects increased, and she was found to have
paracentral scotomas which were thought to be consistent with bilateral
optic neuropathy. Early in November, she was admitted to the hospital
for a course of intravenous methyl prednisolone.

During her hospital stay, she was noted to have short term memory and
speech impairment; her vision did not improve. She was discharged with
the diagnosis of Creutzfeldt-Jakob disease.

Later, the patient developed progressive dementia with marked
impairment of speech and memory. She had complete visual loss,
increased weakness and myoclonus. She died on December 14, 1997.

MF /AV
12/16/97

Patient Name: POULTER, BARBARA
Patient Location: AUTOPSY
Room/Bed:
Printed Date / Time: 01//30/98 - 0832
Page: 2
Continued ....
--------------

Patient Account: 90000014-518
Med. Rec. No.: (0160)118511Q
Patient Name: POULTER, BARBARA
Age: 63 YRS DOB: 10/17/34 Sex: F Race: C
Admitting Dr.:
Attending Dr.:
Date / Time Admitted : 12/14/97 1228

UTMB
University of Texas Medical Branch
Galveston. Texas 77555-0543
(409) 772-1238 Fax (409) 772-5683
Pathology Report

AU-97-00435

GROSS DESCRIPTION:

EXTERNAL EXAMINATION: The body is that of a 63-year-old well-nourished,
well-developed white female. There is no rigor mortis present, and there
is unfixed dependent lividity on the posterior surface. The head is
normocephalic with a moderate amount of gray, medium length scalp hair.
The irides are blue with equal pupils measuring 0.4 mm in diameter. The
nares are patent with no exudate. Dentition is fair. Buccal membranes
are normal. There is normal female hair distribution. The chest does not
have increased anterior-posterior diameter. The abdomen is slightly
protuberant. Lymph node enlargement is not present. The extremities are
unremarkable. The genitalia are those of a normal female. Two
well-healed remote scars are identified in the abdomen: one in the right
upper quadrant and another in the superpubic area.

BRAIN: The brain weighs 1450 gm. The gyri and sulci display a normal
pattern without edema or atrophy. The meninges show no abnormalities.
The circle of Willis, basilar and vertebral arteries show no significant
atherosclerosis. The brain is fixed in formalin for later examination by
a neuropathologist (see neuropathology report). No indentation of the
cingulate gyri, unci or molding of the cerebellar tonsils are noted.

SPINAL CORD: The spinal cord is not removed.

PITUITARY GLAND: The pituitary gland is removed and is fixed in formalin
for subsequent examination by a neuropathologist.

MF /AV
12/16/97


Patient Name: POULTER, BARBARA
Patient Location: AUTOPSY
Room/Bed:
Printed Date / Time: 01/30/98 - 0832

Page 3
Continued ....
--------------

Patient Account : 90000014-518
Med. Rec. No.: (0160)118511Q
patient Name: POULTER, BARBARA
Age: 63 YRS DOB: 10/17/34 Sex: F Race: C
Admitting Dr.:
Attending Dr,:
Date/Time Admitted: 12/14/97 1228

UTMB
University of Texas Medical Branch
Galveston, Texas 77555-0543
(409) 772-1238 Fax (409) 772-5683

Pathology Report AU-97-00435

MICROSCOPIC DESCRIPTION:

BRAIN: Histologic examination of multiple sampled areas of the brain
showed the characteristic features of Creutzfetdt-Jakob disease. These
were present in most sections, but were particularly prominent in the
occipital cortex. The spongiform degeneration was seen in the neuropil
of the gray matter as multiple vacuoles amoung numerous reactive
astrocytes and occasional neuronal cell bodies. These changes were most
notable in the basal layer of the cortex. PAS and amyloid stains will be
performed on selected sections to asses the presence of plaques.

MF /MF
01/28/98

patient Name: POULTER, BARBARA
Patient Location: AUTOPSY
Room/Bed:
Printed Date / Time: 01/30/98 - 0832

Page: 4
Continued ....
--------------

Patient Account: 90000014-518
Med. Rec. No.: (0160}118511Q
Patient Name: POULTER, BARBARA
Age: 63 YRS DOB: 10/17/34 Sex: F Race: C
Admitting Dr.:
Attending Dr.:
Date / Time Admitted : 12/14/97 1228

UTMB
University of Texas Medical Branch
Galveston, Toxas 775550-0543
(409) 772-1238 Fax (409) 772-5683
Pathology Report

Autopsy office (409)772-2858
Autopsy No.: AU-97-00435

FINAL AUTOPSY REPORT

CLINICOPATHOLOGIC CORRELATION:

The clinical findings in this case strongly suggest the diagnosis of
Creutzfeldt-Jakob disease: progressive dementia, typical EEG changes,
visual disturbances and myoclonus. These characteristics indicate this
is a "probable case of CJD", according the criteria set by the EC
Surveillance Group of Creutzfeldt-Jakob Disease in Europe (1).
The definitive diagnosis of Creutzfeldt-Jakob disease, however, is
established by neuropathologic findings. There are three changes that
are classically described and considered diagnoseic: spongiform change,
neuronal loss and astrocytic gliosis. The presence of these can vary
significantly in proportion and distribution and often correlate with
clinical symptoms. This permits classification of the disease into
several variants.

Three variants of Creutzfeldt-Jakob disease have been proposed by Roos
and Gajdusek (2): frontopyramidal, with pyramidal or lower motor neuron
involvement; occipitoparietal {Heidenhain), characterized by disorders
in higher cortical function and vision; and diffuse, with cerebral,
cortical, basal ganglia, thalamic, cerebellar, midbrain and spinal cord
involvement.

Histological examination from multiple samples of the brain in this case
revealed astrocytic gliosis, spongiform degeneration and neuronal loss.
Although these changes were seen in most sections, they were most
prominent in the occipital cortex. This correlates very well with the
clinical history of visual disturbances. Based on this finding, the
present case corresponds to the Heidenhain variant. It is not uncommon
for Creutzfeldt-Jakob disease to present with visual symptoms as the
initial manifestation of the disease. Vargas et al (3) has reported
three cases with these characteristics.

There have been numerous and significant advances in our understanding
of Creutzfeldt-Jakob disease and prion diseases in general. These have
been reviewed in several papers written recently, including one by
Horowich and Weissman (4).

In summary, this 63 year old female with a history of visual
disturbances and dementia of rapid progression was found to have the
neuropathologic changes characteristic of Creutzfeldt-Jakob disease,
predominantly in the occipital cortex. The occipital tropism and
consequent visual symptoms indicate this case corresponds to the
Heidenhain variant.

REFERENCES:

Patient Name: POULTER, BARBARA
Patient location: AUTOPSY
Room/Bed:
Printed Date / Time: 01/30/98 * 0832

Page: 5
Continued ....
--------------

Patient Account: 90000014-518
Med. Rec. No.: (0160)118511Q
Patient Name: POULTER, BARBARA
Age: 63 YRS DOB: 10/17/34 Sex: F Race: C
Admitting Dr.:
Attending Dr.:
Date l Time Admitted : 12/14/97 1228

UTMB
University of Texas Medical Branch
Galveston, Texas 77555-0543
(409) 772-1238 Fax (409} 772-5683
Pathology Report

Autopsy No.: AU-97-00435

FINAL AUTOPSY REPORT

CLINICOPATHOLOGIC CORRELATION:
1. Budka H, et al: Tissue handling in suspected Creutzfeldt-Jakob
disease (CJD) and other human spongiform encephalopathies
(prion diseases), Brain Pathology. 5:319-322,1995.

2. Roos R, Gajdusek DC, Gibbs CJ Jr: The clinical characteristics of
transmissible Creutzfeldt-Jakob disease. Brain 96: 1-20, 1973.

3. Vargas ME, et al: Homonymous field defect as the first
Manifestation of Creutzfeldt-Jakob disease. American Journal of
Ophthalmology. 119:497-504, 1995.

4. Horowich AL, Weissman JS: Deadly conformations - protein misfolding
in prion disease. Cell Vol.89, 499-510, 1997.

MF /MF
01/28/98

SCOT D. PENCIL, M.D., PATHOLOGIST
MARTIN FERNANDEZ, M.D.
01/29/98
(Electronic Signature)


Patient Name: POULTER, BARBARA
Patient Location: AUTOPSY
Room/Bed:
Printed Date / Time: 01/30/98 - 0832

Page: 6
END OF REPORT
--------------

The University of Texas Medical Branch at Galveston

Gerald A, Campbell, Ph.D., M.D,
Associate Professor and Director
Division of Neuropathology
Department of Pathology

February 26, 1998

Pieduigi Gambetti, M.D.
Professor
Institute of Pathology
Case Western Reserve University
2085 Adelbert Road
Cleveland Ohio 44106

Dear Dr, Gambetti:

Enclosed please find the microscopic slides and autopsy report from our
patient, Barbara Poulter (Hosp.# 11851lQ, Autopsy # AU97-435). These
slides are being sent for consultation at the request of Mr. Singletary,
Ms. Poulter's son and next of kin. We will also send frozen tissue from
the brain on dry ice next week, and someone will call you on the day
the tissue is shipped. Please return the slides when you have finished
with your examination. If you need any further information, please do
not hesitate to call me. Thanks for your assistance with this case.

Sincerely,
Gerald A. Campbell
------------------
CASE WESTERN RESERVE UNIVERSITY

February 26, 1988

Gerald Campbell, M.D,, PhD.
Division of Neuropathology, G85
University TX Medical Branch
Galveston, TX 77555-0785

Dear Dr. Campbell,

As per our telephone conversation concerning a recent case of CJD, I
Will be willing to examine slides and the frozen tissue on western
blotting, I will issue a report to you about our conclusions. Below is
my address, Our Fed Ex number is XXXXXXXXXXXXXXX.

Thank your for your assistance in this matter,

Best personal regards,

Pierluigi Gambetti, M.D.

PG:In

Division of Neuropathology
Pierluigi Gambetti, M.D. Director
Institute Of Neuropathology
2085 Adelbert Road
Cleveland, Ohio 44106

Phone 216-368-0587
Fax 216-368-2546
------------------
CASE WESTERN RESERVE UNIVERSITY

February 27, 1998

Dr. Gerald A. Campbell
The University of Texas
Medical Branch at Galveston
Division of Neuropathology, G85
Galveston. TX 77555-0785

Dear Dr. Campbell,

We are in receipt of the slides you sent on Mrs. Barbara Poulter (your
#: AU97-435;our#098-28).

Best personal regards,
Pierluigi Gambetti, M.D.

PG:sb

Division of Neuropathology
Pierluigi Gambetti, M.D., Director
-----------------------------------
CASE WESTERN RESERVE UNIVERSITY

March 30, 1998

Dr. Gerald A, Campbell
The University of Texas
Medical Branch at Galveston
Division of Neuropathology
Department of Pathology
Galveston, Texas

Dear Dr Campbell,

We performed Western immunoblot analysis on the frozen tissue from your
case #AU97-435 (our #098-28). The Immunoblot reveals the presence of
protease-resistant prion probein (PrPres) confirming the diagnosis of
prion disease. The immunoblot pattern of PrPres is consistent with the
diagnosis of Creutzfeldt-Jakob disease.

Thank you for referring to us this interesting case.

Sincerely,

Piero Parchi, M.D.

Pierluigi Gambetti, M.D.

PP:sb

Division of Neuropathology
Pierluigi Gambetti, M.D., Director
Case Western Reserve University

This Autopsy report is for the use of anyone, who is trying to
understand this hideous disease CJD. I hope it can be benificial for
some in researching human TSE. Please remember, this was my Mom, and
to use this with great respect.

thank you,
kind regards,

Terry S. Singeltary Sr., Bacliff, Texas USA

-------------------------------
From: Jeff (webwizard.vegsource.org)
Subject: Very interesting letter from son of CJD victim -- and
alleged connection to cows

Date: April 22, 1998 at 19:53:42 EST

This was sent to Oprah Winfrey, reprinted here by permission:

I am the madson of a deadmom who died of madcow.(heidenhain variant
creutzfeldt-jacob disease.) I sat with her for 10 weeks and watched
as this hidious disease ate her brain up. She wrote in her journal
that she started to see brown spots on sept. 27, 1997. These were her
first symptoms -- apprx.10 days later she was blind, about 2 weeks
later she had lost control of her coordination, walking, and speech.

She would get these uncontrolable jerks that at
times would take 3 of us to hold her down. Around the
8th week she was totally bedridden. She died in the
10th week on 12-14-97. THANK GOD!

If you ever see this disease, as I did with my mom,
you will truly believe that madcow is here. I truely
believe that is what my mom died of. They can call it
what ever they want to.

Now, I will take this a step further. My neighbor's
mother also died of c.j.d. She died on 12-14-96, they
had diagnosed it as Alzheimers, until the autopsy he demanded
ruled out alzheimers and ruled in c.j.d.

About a month ago my neighbor called me over, he had
been going through some old boxes of his mom's
and came across some pills he thought I should see.
When I read the ingredients I just about sh*t!

INGREDIENTS: vacuum dried bovine brain, bone meal,
bovine eye, veal bone, bovine liver powder,
bovine adrenal, vacuum dried bovine kidney, and
vacuum dried porcine stomach. It was a cow in a pill!
This woman taking these pills died of c.j.d. Could
it be madcow in a pill?

I called the texas dept. of health (T.D.H.) the
next day, and the following day they were out here and
got the pills. I had located the manufacture and
called with a bogus story and a list of doctors that
would prescribe them in houston. The T.D.H. called a
few days later, asking for the list of doctors, their
phone numbers, and told me they would take it from there.
I need not persue it any further!

Not to long ago, 4 or 5 weeks, a girl showed up at
my door. She had called crying a week earlier and
could not talk. She had seen a story on T.V. about
my mother. Anyway, when I first saw her I knew she
had seen it too (madcow). Her mother had died of
c.j.d. on 2-14-97.

This disease is here and you can call it what ever you
want, c.j.d., n.v.c.j.d., hvCJD, b.s.e. or madcow, for
what it is. But, that young man who died of n.v.c.j.d.
in England, Steve Churchhill, had the exact same
symptoms as my mother. There is also a girl in Ft. Worth
Texas who called me. She had seen an article
about my mom in the dallas morning news. Her dad had died
of c.j.d. so far we have come up with about
18 people who has died of c.j.d. in texas, 15 confirmed.
I have heard from other people its up to 32.

I am tired of hearing this crap about nv-cjd being
in just young people. That same old line about how
nv-cjd victims are much younger and their clinical
course from first sign of symtoms to death is much
longer. Any diseases clinical course is going to be
longer in younger people, because their body and
organs are much younger and healthier. But, in the
end, their brains are full of spongiform holes, just like
the older folks. Just because the plaques are more
extreme, does not mean its a different disease. Could
it not be just a more extreme case of typical c.j.d.????

Greed is what it is all about. They banned feeding
cattle to cattle. But, are still allowed to feed those
downer cows to pork and poultry. Then they are still
allowed to feed the pork and poultry byproducts
back to the cows. Now Dr. Gibbs writes that the
prion-protien can survive the digestinal track and
composting process. So the prion-protein goes right
back to the cow. We must ban feeding all animals to
animals. Its just an endless cycle of greed thats
killing people.

I have requested that further test be done on my moms
brain.(frozen tissue, paraffeine sections and
serum) be sent to case western reserve university in
Cleveland, Ohio. Dr. Pierre Lugi Gambetti.

I hope you find some interest in this. I just don't
believe we are being told everything. The gov. lied
about asbestos for 75 years.

P.S.-- the results from Case Western Reserve Universitiy,
on my Mothers Brain, came back positive for
the prion protein PrPres, confirming the prion disease.........

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

From: TSS ()
Subject: Re: Abnormal PrP in the retina of the most commonly subtype sCJD
Date: August 22, 2005 at 6:59 am PST


1: Rev Neurol (Paris). 2005 May;161(5):578-81.


[Heidenhain's variant of Creutzfeldt-Jakob's disease]

[Article in French]

Fauquembergue M, Tilikete C, Perret-Liaudet A, Kopp N, Krolak-Salmon P,
Vighetto A.

Unite de Neuro-ophtalmologie et service de Neurologie D, Hospices Civils de
Lyon, Hopital Neurologique Pierre-Wertheimer, Bron.

INTRODUCTION: Creutzfeldt-Jakob's disease has various anatomoclinical
presentations including a rare form with preponderant visual signs described
by Heidenhain. In this form, the visual symptoms may be isolated for a few
weeks, leading to multiple ophthalmological examinations. OBSERVATION: We
report the case of a 75-year-old woman who developed isolated visual
disorders which rapidly increased over a period of two months. Addition of
neurological symptoms, abnormalities of EEG and positivity of 14-3-3 protein
led to the diagnosis of Creutzfeldt-Jakob's disease. The patient died 14
months after the first neuroophthalmologic signs. The diagnosis was
established by post-mortem examination and immuno-electrophoretic
demonstration of type 1 prion protein. CONCLUSION: Heidenhain's form of
Creutzfeldt-Jakob's disease highlights the importance of general rules for
prevention of iatrogenic hazard during ophthalmological examinations.

PMID: 16106810 [PubMed - in process]
http://www.ncbi.nlm.nih.gov/

TSS


----- Original Message -----
From: "Terry S. Singeltary Sr."
To:
Sent: Friday, August 19, 2005 10:00 PM
Subject: Abnormal prion protein in the retina of the most commonly occurring
subtype of sporadic Creutzfeldt-Jakob disease


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

CJD WATCH MESSAGE BOARD
TSS
Abnormal PrP in the retina of the most commonly subtype sCJD
Fri Aug 19, 2005 22:00
68.238.104.92


SCIENTIFIC REPORT

Abnormal prion protein in the retina of the most commonly occurring subtype
of sporadic Creutzfeldt-Jakob disease
M W Head1, A H Peden1, H M Yull1, D L Ritchie1, R E Bonshek2, A B Tullo2 and
J W Ironside1
1 National CJD Surveillance Unit, University of Edinburgh, Western General
Hospital, Edinburgh EH4 2XU, UK
2 Academic Department of Ophthalmology, Manchester Royal Eye Hospital,
Manchester M13 9WH, UK


Correspondence to:
Dr M W Head
National CJD Surveillance Unit, Bryan Matthews Building, Western General
Hospital, Edinburgh EH4 2XU, UK; m.w.head@ed.ac.uk

ABSTRACT
Background: Involvement of the eye has been reported in patients with
variant Creutzfeldt-Jakob disease (vCJD), but there is disagreement on
whether retinal involvement occurs in sporadic Creutzfeldt-Jakob disease
(sCJD).

Methods: Western blotting, paraffin embedded tissue blotting, and
immunohistochemistry were used to test whether the abnormal form of the
prion protein (PrPSc) accumulates to detectable levels in the eye in a case
of the most common subtype of sCJD (MM1).

Results: Low levels of PrPSc were detectable in the retina, localised to the
plexiform layers of the central retina. PrPSc was not detectable in other
ocular tissues.

Conclusions: The abnormal form of the prion protein is present in the retina
in the most common sCJD subtype (MM1), albeit at levels lower than those
found previously in vCJD and in sCJD of the VV2 subtype.

----------------------------------------------------------------------------
----

Abbreviations: sCJD, sporadic Creutzfeldt-Jakob disease; vCJD, variant
Creutzfeldt-Jakob disease


Keywords: Creutzfeldt-Jakob disease; prion protein; PRNP codon 129 genotype;
retina

http://bjo.bmjjournals.com/


HARD telling how many people my mother exposed with all the exams she had during her first symptoms of blindness while being examinded with many different optical equipment, during her short course of clinical disease that being from onset of symptoms to death approx. 10 weeks. ...


kind regards,
Terry S. Singelary Sr.
Bacliff, Texas USA

TSS




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