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From: TSS ()
Subject: Creutzfeldt-Jakob Disease Presenting with Confusion and Visual Disturbance and hvCJD i.e. Heidenhain Variant CJD case report
Date: July 18, 2007 at 12:31 pm PST

TO The Editor-in-Chief,

Archives of Iranian Medicine, Editorial Office,

Academy of Medical Sciences of I.R.Iran,

P.O. Box: 19395-4655, Tehran, Iran.

TSS submission to the following study, comments and submission to follow ;

Arch Iranian Med 2007; 10 (3): 397 – 400
Archives of Iranian Medicine, Volume 10, Number 3, July 2007 397

Creutzfeldt-Jakob Disease Presenting with Confusion and Visual Disturbance

Masoud Nikanfar MD*, Mehdi Farhoudi MD•**, Monireh Halimi MD***,
Fereidoon Ashrafian-Bonab MD***, Kaveh Mehrvar MD†

Creutzfeldt-Jakob disease is increasingly being reported in the last three
decades as a result
of increased awareness for the disease. Various studies have reported an
annual incidence
of 0.5 – 1.5 cases of Creutzfeldt-Jakob disease per million of general
However, in our country, like other developing countries, the disease is
still under-reported.
Herewith, we described our clinical experience with an autopsy-proven case
of Creutzfeldt-Jakob

Archives of Iranian Medicine, Volume 10, Number 3, 2007: 397 – 400.


Prions can cause neurodegenerative
diseases that have long incubation
periods and progress inexorably once the
clinical symptoms appear. So far, five human prion
diseases have been recognized including Kuru,
Creutzfeldt-Jakob disease (CJD), variant
Creutzfeldt-Jakob disease (vCJD also known as
new variant CJD), Gerstmann-Straussler-Scheinker
(GSS) syndrome, and fatal familial insomnia
(FFI).1, 2 Bovine spongiform encephalopathy
(BSE), one of prion infections affecting animals,
was responsible for a more widespread public
attention with its possible link to vCJD.3, 4
These human prion diseases share certain
common neuropathologic features including
neuronal loss, proliferation of glial cells, absence
of an inflammatory response, and the presence of
small vacuoles within the neutrophils, which
produces a spongiform appearance. The current
theory is that prion diseases are associated with the
accumulation of an abnormal form of a host cell
protein, the so-called “prion protein” (PrP).5
This paper describes the clinical experience
with an autopsy-proven case of CJD managed at
the Department of Neurology of Razi Hospital,
Tabriz, North-West of Iran.

Case Report

A 71-year-old woman referred to our hospital
on May 3, 2004 with a one and a half-month
history of visual symptoms accompanied by
transient confusion and disorientation and dystonic
posture in her right arm.
Her family members reported that she had
transient attacks of agitation, hallucinations, and
confusional state with a duration of about 15
minutes. After each attack, she became normal
without any memories of attacks. Gradually, the
frequency of these attacks increased. Meanwhile,
she complained of transient visual problems such
as macropsy, micropsy, blurred vision, and color
vision disturbance. She had also transient dystonic
posture in her right arm. Before admission, she
also developed a mild left-sided hemiparesis. She
was visited by a psychiatrist who prescribed
haloperidol and clonazepam with no beneficial
effect; her condition became worse progressively.
On admission, she had mild confusion, and
headache, and vertigo. General examination was
normal. In neurologic examination, she had a left
hemiparesis, left central hemifacial weakness, no
in her right arm.
All biochemical laboratory tests of plasma and
urine were normal. Cerebrospinal fluid analysis
was normal. In brain computed tomography (CT)
and magnetic resonance imaging (MRI), multiple
lacunar infarctions and senile atrophy were
reported. In her first electroencephalogram (EEG),
generalized slow sharp waves appeared in all
montages (Figure 1A).
The patient’s general condition and level of
consciousness progressively deteriorated during
hospitalization. After 15 days, she developed
myoclonie seizures — first in her right arm and
then generalized. In the next EEG, there was
progressive slowness and periodic sharp waves. In
her last EEG, slow and disorganized background
activity that was interrupted by repetitive
discharges of large sharp waves in all montages —
about one cycle per second — were recorded
(Figure 1B, C). She was intubated and finally on
June 9, 2004, she died of sepsis.
In pathologic study performed two days after
death, the spongiform encephalopathy was
documented. Histopathologic examination revealed
no Kuru plaque in Congo red and PAS staining. No
inflammatory infiltration was present (Figure 2).

snip...full text ;

Greetings Editor-in-Chief et al Archives of Iranian Medicine, Editorial

I kindly wish to submit the following to the Authors of the above study, and
the Archives of Iranian Medicine, Editorial Office, Academy of Medical
Sciences of I.R.Iran.

THESE symptoms are very similar of which my mother had i.e. Heidenhain
Variant Creutzfeldt Jakob Disease. I can remember her and her right arm,
and she would stretch her arm against the wall, ...... strange reading this.
also, clinical onset of disease to death similar, approx. 12+ weeks, and of
course my mother went blind at onset, again, with similar symptoms.
ODD, they don't even mention the Heidenhain Variant of CJD, one of
six documented phenotypes of the sporadic CJDs to date, with 'unknown'
phenotype of Sporadic CJD growing in USA (14 or 15 persons which display
selectively SPRPSC and practically no detected RPRPSC proteins). ...TSS

The Heidenhain Variant of Creutzfeldt-Jakob Disease

Stefan Kropp, MD; Walter J. Schulz-Schaeffer, MD; Michael Finkenstaedt, MD;
Christian Riedemann, MD; Otto Windl, PhD; Bernhard J. Steinhoff, MD; Inga
Zerr, MD; Hans A. Kretzschmar, MD; Sigrid Poser, MD

Arch Neurol. 1999;56:55-61.

Objective To investigate whether typical neuropathological and radiological
findings can be identified in patients with the clinical diagnosis of the
Heidenhain variant of Creutzfeldt-Jakob disease (CJD).

Design Case study. The clinical symptoms, neuropathological findings,
electroencephalograms, magnetic resonance images, and cerebrospinal fluid
samples of 14 Heidenhain cases were evaluated. Neuropathological changes
were compared with those in a group of 14 patients with ataxia as the
leading clinical sign.

Setting A university hospital, base of the German National
Creutzfeldt-Jakob Disease Surveillance Study.

Patients Medical records of 169 neurologically examined patients with
prospectively classified and neuropathologically confirmed CJD were

Main Outcome Measure Difference in neuropathological and radiological
findings between patients with the Heidenhain variant and other patients
with CJD.

Results Of 169 patients with confirmed CJD, 20% showed characteristic
clinical findings such as blurred vision, visual field restriction,
metamorphopsia, or cortical blindness. Disease course of the Heidenhain
group, as compared with the group of all patients with definite CJD, was
significantly shorter (5.7 months vs 7.5 months; P=.02, t test).
Neuropathological examination of patients with the Heidenhain variant showed
most pronounced changes in the occipital lobe but less damage in the
cingulate gyrus and basal ganglia compared with 14 patients with CJD who had
ataxia as the leading clinical sign. Eleven (92%) of 12 genetically analyzed
Heidenhain cases were homozygous for methionine at codon 129 of the prion
protein gene (PRNP). In 9 of 9 cases, the 14-3-3 protein was present. In 7
(78%) of 9 cases, the level of neuron-specific enolase was elevated, with a
concentration above 35 ng/mL. Periodic sharp-wave complexes were observed in
11 (78%) of the 14 cases. In 7 (63%) of 11 patients, magnetic resonance
images showed symmetric hyperintensities in the basal ganglia in the T2- and
proton-weighted sequence. In 4 of 11 cases the T2- and proton
density–weighted images showed a pronounced signal increase confined to the
gray matter of the occipital and visual cortex. Isolated atrophy of the
visual cortex was noticeable in 2 of 11 cases.

Conclusions The clinical presentation of the Heidenhain variant of CJD was
shown to correlate with the neuropathological findings of gliosis and nerve
cell loss. In patients with visual disorders of unclear origin and signs of
dementia, the differential diagnosis of a Heidenhain variant of CJD must be
taken into consideration.

From the Departments of Neurology (Drs Kropp, Riedemann, Zerr, and Poser),
Neuropathology (Drs Schulz-Schaeffer, Windl, and Kretzschmar),
Neuroradiology (Dr Finkenstaedt), and Clinical Neurophysiology (Dr
Steinhoff), Georg-August-University Göttingen, Göttingen, and the MR/CT
Institute Hamburg, Hamburg (Dr Finkenstaedt), Germany.

full text pdf ;

Of Illusions, Hallucinations and Creutzfeldt-Jakob Disease (Heidenhain’s

Creutzfeldt-Jakob Disease Presenting with Visual Blurring, Diplopia and
Visual Loss: Heidenhain’s Variant

K E Lee,*MBBS, MRCP (UK), N K Loh,**MBBS, MRCP (UK), M Med (Int Med), A K Y
Tan,***FAMS, MBBS, MRCP (UK), W L Lee,†
MBBS (Hons), MRCP (UK), M Med (Paed), H T L Tjia,‡
FAMS, MBBS, M Med (Int Med)

Heidenhain Variant Creutzfeldt Jakob Disease autopsy case report 'MOM'

University of Texas Medical Branch
114 McCullough Bldg.
Galveston, Texas 77555-0785


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):



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 telephone 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:

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

Autopsy' Office (409)772-2858

Autopsy NO.: AU-97-00435

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


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 Location: AUTOPSY
Printed Date; Time: 01/30/98 - 0832

Page: 1
Continued ....

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

Pathology Report

Autopsy NO,: AU-97-00435

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 corresponding 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.

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.

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 amyloid evident in the special stains.
no evidence of plaques.GAE

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

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

Patient Location: AUTOPSY
Printed Date: Time: 02/09/98 - 1120

Page 2

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


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 defects 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

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 otherwise 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 the 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 is 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
throughout most of the brain, except for the inferior and 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. The 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.


Page 1
Continued ....

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

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

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

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


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

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.


Patient Location: AUTOPSY
Printed Date / Time: 01//30/98 - 0832
Page: 2
Continued ....

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

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



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.


Patient Location: AUTOPSY
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

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

Pathology Report AU-97-00435


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.


Patient Location: AUTOPSY
Printed Date / Time: 01/30/98 - 0832

Page: 4
Continued ....

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

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

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



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 diagnostic: 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

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.


Patient location: AUTOPSY
Printed Date / Time: 01/30/98 * 0832

Page: 5
Continued ....

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

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

Autopsy No.: AU-97-00435



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.


(Electronic Signature)

Patient Location: AUTOPSY
Printed Date / Time: 01/30/98 - 0832

Page: 6

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

Pierluigi Gambetti, M.D.
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.

Gerald A. Campbell

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.


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

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.


Division of Neuropathology
Pierluigi Gambetti, M.D., Director

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 protein (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.


Piero Parchi, M.D.

Pierluigi Gambetti, M.D.


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 beneficial 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



DIED 12-14-97

If I had one last thing I could tell you, it would be, I love you. I'm sorry
for the stupid argument we had the last few months, BEFORE this hideous
disease ROARED through your body. BUT, I PROMISE MOM,


We got a call from my Mother around the end of Oct. saying "the damn'est
thing has happened, I can't see, and if I'm talking to you and I don't make
sense, bare with me, I'll come back". It was a shock to all of us. It seems
that a few days before, she was crossing the ferry and became frightened
because she was having problems seeing. She explained it as looking down a
tunnel or not being able to see from the sides, and seeing brown spots.

We had NOT been talking, over something, we had NO control of, for a few
months. So I did not know she had been having these visual problems, until
she was blind. These were her first symptoms. From that point on, I was with
her most everyday. I had to cross the Galveston/Bolivar ferry, and its about
30 minutes each way, so as the disease progressed, it gave me a great deal
of time to think. When the visual problems started, it was about 2 weeks
later, and she was blind. That led to coordination, and balance problems
starting. But as this hideous disease progresses, it just GOES. You don't
seem to catch up with it. It was like a fire in a hurricane. We would go out
and get her things she needed one day, and the next day it would be
obsolete, because the disease had gone to another stage. So you started
over. Her coordination and balancing led to being in a wheel-chair. She was
starting to get these trembles. I also noticed how her hands and feet
started to go inward. Her speech was nothing more than jerble at this time,
and this was probably about the 6th week, (at this point we had to tie her
to the wheel chair, to keep her from falling out). The trembles had turned
into SEVERE JERKS, that at times would take 3 of us to hold her down. I will
never forget that....About her 8th week she became comatose....She died
around the 10th week. I had spent the night, she had problems through the
night, so the nurse came. She checked her out and comforted us, (HOSPICE IS
A WONDERFUL ORGANIZATION). The nurse said she seemed to be alright and that
it would probably be alright to go home for a few hours. I was on the Ferry,
going back to Galveston, when I got the call, she was gone. What can you do,
Mom was gone, and I was stuck on the Damn Ferry, going the wrong direction.

She knew what she had. I remember, before she had lost her speech
completely. After a doctors conference, and CJD had come up. She heard us
say CJD, and she screamed, SHE knew! At that point, I didn't know what was,
much less, CREUTZFELDT JAKOB DISEASE.....I have learned a lot since. I have
learned I truly miss my Mom and I am MAD as hell that she is gone!






From: Jeff (
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 hideous 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 uncontrollable 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 truly
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 pursue 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 symptoms 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 University,
on my Mothers Brain, came back positive for
the prion protein PrPres, confirming the prion disease.........

kind regards,

Terry S. Singelary Sr.
P.O. Box 42
Bacliff, Texas USA



HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD
only theory

TSEs have been rampant in the USA for decades in many species, and they all
have been rendered and fed back to animals for human/animal consumption. I
propose that the current diagnostic criteria for human TSEs only enhances
and helps the spreading of human TSE from the continued belief of the
UKBSEnvCJD only theory in 2007. With all the science to date refuting it, to
continue to validate this myth, will only spread this TSE agent through a
multitude of potential routes and sources i.e. consumption, surgical, blood,
medical, cosmetics etc. I propose as with Aguzzi, Asante, Collinge, Caughey,
Deslys, Dormont, Gibbs, Ironside, Manuelidis, Marsh, et al and many more,
that the world of TSE Transmissible Spongiform Encephalopathy is far from an
exact science, but there is enough proven science to date that this myth
should be put to rest once and for all, and that we move forward with a new
classification for human and animal TSE that would properly identify the
infected species, the source species, and then the route. This would further
have to be broken down to strain of species and then the route
of transmission would further have to be broken down. Accumulation and
Transmission are key to the threshold from subclinical to clinical disease,
and of that, I even believe that physical and or blunt trauma may play a
role of onset of clinical symptoms in some cases, but key to all this, is to
stop the amplification and transmission of this agent, the spreading of, no
matter what strain. BUT, to continue with this myth that the U.K. strain of
BSE one strain in cows, and the nv/v CJD, one strain in humans, and that all
the rest of human TSE is one single strain i.e. sporadic CJD (when to date
there are 6 different phenotypes of sCJD), and that no other animal TSE
transmits to humans, to continue with this masquerade will only continue to
spread, expose, and kill, who knows how many more in the years and
decades to come. ONE was enough for me, My Mom, hvCJD, DOD 12/14/97
confirmed, which is nothing more than another mans name added to CJD, like
CJD itself, Jakob and Creutzfeldt, or Gerstmann-Straussler-Scheinker
syndrome, just another CJD or human TSE, named after another human. WE are
only kidding ourselves with the current diagnostic criteria for human and
animal TSE, especially differentiating between the nvCJD vs the sporadic CJD
strains and then the GSS strains and also the FFI fatal familial insomnia
strains or
the ones that mimics one or the other of those TSE? Tissue infectivity and
typing of the many variants of the human and animal TSEs are paramount in
all variants of all TSE. There must be a proper classification that will
differentiate between all these human TSE in order to do this. With the CDI
and other more sensitive testing coming about, I only hope that my proposal
will some day be taken seriously.

My name is Terry S. Singeltary Sr. and I am no scientist, no doctor and have
no PhDs, but have been independently researching human and animal TSEs since
the death of my Mother to the Heidenhain Variant of Creutzfeldt Jakob
Disease on December 14, 1997 'confirmed'. ...END

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

Singeltary, Sr et al. JAMA.2001; 285: 733-734.





MARCH 26, 2003

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob

disease in the United States

Email Terry S. Singeltary:

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to

comment on the CDC's attempts to monitor the occurrence of emerging

forms of CJD. Asante, Collinge et al [1] have reported that BSE

transmission to the 129-methionine genotype can lead to an alternate

phenotype that is indistinguishable from type 2 PrPSc, the commonest

sporadic CJD. However, CJD and all human TSEs are not reportable

nationally. CJD and all human TSEs must be made reportable in every

state and internationally. I hope that the CDC does not continue to

expect us to still believe that the 85%+ of all CJD cases which are

sporadic are all spontaneous, without route/source. We have many TSEs in

the USA in both animal and man. CWD in deer/elk is spreading rapidly and

CWD does transmit to mink, ferret, cattle, and squirrel monkey by

intracerebral inoculation. With the known incubation periods in other

TSEs, oral transmission studies of CWD may take much longer. Every

victim/family of CJD/TSEs should be asked about route and source of this

agent. To prolong this will only spread the agent and needlessly expose

others. In light of the findings of Asante and Collinge et al, there

should be drastic measures to safeguard the medical and surgical arena

from sporadic CJDs and all human TSEs. I only ponder how many sporadic

CJDs in the USA are type 2 PrPSc?

Copyright © 2003 Published by Elsevier Ltd.

Tracking spongiform encephalopathies in North America

Xavier Bosch

Available online 29 July 2003.

Volume 3, Issue 8, August 2003, Page 463

“My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my
mom to hvCJD (Heidenhain variant CJD)
and have been searching for answers ever since. What I have found is that we
have not been told the truth. CWD
in deer and elk is a small portion of a much bigger problem.”



sporadic CJD in farmers with BSE cows


18 January 2007 - Draft minutes of the SEAC 95 meeting (426 KB) held on 7
December 2006 are now available.


64. A member noted that at the recent Neuroprion meeting, a study was
presented showing that in transgenic mice BSE passaged in sheep may be more
virulent and infectious to a wider range of species than bovine derived BSE.

Other work presented suggested that BSE and bovine amyloidotic spongiform
encephalopathy (BASE) MAY BE RELATED. A mutation had been identified in the


3:30 Transmission of the Italian Atypical BSE (BASE) in Humanized Mouse

Models Qingzhong Kong, Ph.D., Assistant Professor, Pathology, Case Western

Bovine Amyloid Spongiform Encephalopathy (BASE) is an atypical BSE strain
discovered recently in Italy, and similar or different atypical BSE cases
were also reported in other countries. The infectivity and phenotypes of
these atypical BSE strains in humans are unknown. In collaboration with
Pierluigi Gambetti, as well as Maria Caramelli and her co-workers, we have
inoculated transgenic mice expressing human prion protein with brain
homogenates from BASE or BSE infected cattle. Our data shows that about half
of the BASE-inoculated mice became infected with an average incubation time
of about 19 months; in contrast, none of the BSE-inoculated mice appear to
be infected after more than 2 years.

***These results indicate that BASE is transmissible to humans and suggest
that BASE is more virulent than
classical BSE in humans.***

6:30 Close of Day One

1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype
of 'UNKNOWN' strain growing. ...

There is a growing number of human CJD cases, and they were presented last
week in San Francisco by Luigi Gambatti(?) from his CJD surveillance

He estimates that it may be up to 14 or 15 persons which display selectively
SPRPSC and practically no detected RPRPSC proteins.

[Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk
Materials for Human Food and Requirement for the Disposition of
Non-Ambulatory Disabled Cattle

Terry S. Singeltary

Page 1 of 17

Sent: Monday, July 24, 2006 1:09 PM

To: FSIS RegulationsComments

Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine
Spongiform Encephalopathy (BSE)

Docket No, 04-047-l Regulatory Identification No. (RIN) 091O-AF46 NEW BSE
SAFEGUARDS (comment submission)


Docket No. 2003N-0312 Animal Feed Safety System [TSS SUBMISSION]

Docket Management Docket: 02N-0273 - Substances Prohibited From Use in

Animal Food or Feed; Animal Proteins Prohibited in Ruminant Feed

Comment Number: EC -10

Accepted - Volume 2



File Format: PDF/Adobe Acrobat -

Page 1. J Freas, William From: Sent: To: Subject: Terry S. Singeltary

Sr. [] Monday, January 08,200l 3:03 PM freas ...

Asante/Collinge et al, that BSE transmission to the 129-methionine

genotype can lead to an alternate phenotype that is indistinguishable

from type 2 PrPSc, the commonest _sporadic_ CJD;

Docket Management Docket: 96N-0417 - Current Good Manufacturing Practice
in Manufacturing, Packing, or Holding Dietary Ingredients a
Comment Number: EC -2
Accepted - Volume 7

[PDF] Appendices to PL107-9 Inter-agency Working Group Final Report 1-1
File Format: PDF/Adobe Acrobat - View as HTML
Agent, Weapons of Mass Destruction Operations Unit Federal Bureau of
those who provided comments in response to Docket No. ...
Meager 8/18/01 Terry S. Singeltary Sr ...

# Docket No: 02-088-1 RE-Agricultural Bioterrorism Protection Act of 2002;
TSS 1/27/03 (0)

Docket Management

Docket: 02N-0276 - Bioterrorism Preparedness; Registration of Food
Facilities, Section 305
Comment Number: EC-254 [TSS SUBMISSION]

Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518

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