Tuesday, July 29, 2008

Heidenhain Variant Creutzfeldt Jakob Disease Case Report

hvCJD has been around a long time, so have different strains of TSE, cwd, scrapie, and some strain of TSE in cattle here in the USA, if you go by sound science that was ignored ;

Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME. Since previous incidences of TME were associated with common or shared feeding practices, we obtained a careful history of feed ingredients used over the past 12-18 months. The rancher was a "dead stock" feeder using mostly (>95%) downer or dead dairy cattle and a few horses. Sheep had never been fed.



http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf



sporadic CJD is not a single strain, but multiple strains of human TSE i.e. sporadic CJD, that to date, the route and source has not been confirmed.


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 population. 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 disease.

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

Introduction

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). Discussion

snip...full text ;



http://www.ams.ac.ir/AIM/07103/0023.pdf



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

I kindly wish to submit the following to the Authors of the above study, and to 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 analyzed.

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.



http://archneur.ama-assn.org/cgi/content/abstract/56/1/55



full text pdf ;



http://archneur.ama-assn.org/cgi/reprint/56/1/55.pdf



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



http://neuro.psychiatryonline.org/cgi/reprint/17/1/124.pdf



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)



http://www.annals.edu.sg/pdf_nov98/leeke.pdf




Heidenhain Variant Creutzfeldt Jakob Disease autopsy case report 'MOM'



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 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:

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 Patient Location: 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 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.

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 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 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 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

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 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.

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, Texas 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 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 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 / 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

Pierluigi 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 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.

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 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


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


BARBARA FREDERICK POULTER

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, YOUR DEATH WILL NOT GO UNANSWERED!

HEIDENHAN VARIANT CREUTZFELDT JAKOB DISEASE

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!

Terry/MADSON!!!

SYMPTOMS:

VISION - BLIND IN ABOUT 10 TO 14 DAYS

COORDINATION AND MUSCLE CONTROL SWALLOWING DIFFICULTY CONFUSION AND DEMENTIA SPEECH PROBLEMS HALLUCINATIONS TREMBLES TOO SEVERE JERKING LOSS OF WEIGHT HANDS AND FEET GREW INWARD UPPER TRUNK STIFFNESS, SHOULDER, UPPER ARM



http://www.fortunecity.com/healthclub/cpr/798/terry.htm




Back to MANY FACES OF CJD



http://www.fortunecity.com/healthclub/cpr/798/cjd.htm




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 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



================================================



-------- Original Message -------- Subject: re-BSE prions propagate as
either variant CJD-like or sporadic CJD Date: Thu, 28 Nov 2002 10:23:43
-0000 From: "Asante, Emmanuel A" To: "'flounder@wt.net'"


Dear Terry,

I have been asked by Professor Collinge to respond to your request. I am
a Senior Scientist in the MRC Prion Unit and the lead author on the
paper. I have attached a pdf copy of the paper for your attention. Thank
you for your interest in the paper.

In respect of your first question, the simple answer is, yes. As you
will find in the paper, we have managed to associate the alternate
phenotype to type 2 PrPSc, the commonest sporadic CJD.
It is too early to be able to claim any further sub-classification in
respect of Heidenhain variant CJD or Vicky Rimmer's version. It will
take further studies, which are on-going, to establish if there are
sub-types to our initial finding which we are now reporting. The main
point of the paper is that, as well as leading to the expected new
variant CJD phenotype, BSE transmission to the 129-methionine genotype
can lead to an alternate phenotype which is indistinguishable from type
2 PrPSc.

I hope reading the paper will enlighten you more on the subject. If I
can be of any further assistance please to not hesitate to ask. Best wishes.

Emmanuel Asante
<> ____________________________________
Dr. Emmanuel A Asante MRC Prion Unit & Neurogenetics Dept. Imperial
College School of Medicine (St. Mary's) Norfolk Place, LONDON W2 1PG
Tel: +44 (0)20 7594 3794 Fax: +44 (0)20 7706 3272 email:
e.asante@ic.ac.uk (until 9/12/02)
New e-mail: e.asante@prion.ucl.ac.uk (active from now)
____________________________________

2008
10 people killed by new CJD-like disease

Public release date: 9-Jul-2008

Since Gambetti's team wrote a paper describing an initial 11 cases referred to his centre between 2002 and 2006 (Annals of Neurology, vol 63, p 697), another five have come to light. "So it is possible that it could be just the tip of the iceberg," Gambetti says.

snip...end



http://www.eurekalert.org/pub_releases/2008-07/ns-tpk070908.php




sporadic CJD, the big lie

Thursday, July 10, 2008 A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease update July 10, 2008



http://cjdmadcowbaseoct2007.blogspot.com/2008/07/novel-human-disease-with-abnormal-prion.html




Thursday, July 10, 2008 A New Prionopathy update July 10, 2008



http://cjdmadcowbaseoct2007.blogspot.com/2008/07/new-prionopathy-update-july-10-2008.html




MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L, and or Italian L-BASE



http://bse-atypical.blogspot.com/2008/03/mad-cow-disease-terminology-uk-c-bse.html




Saturday, June 21, 2008 HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory JUNE 2008



http://cjdmadcowbaseoct2007.blogspot.com/2008/06/human-and-animal-tse-classifications-ie.html




http://www.usda.gov/oig/webdocs/50601-10-KC.pdf




Communicated by: Terry S. Singeltary Sr.

[In submitting these data, Terry S. Singeltary Sr. draws attention to the steady increase in the "type unknown" category, which, according to their definition, comprises cases in which vCJD could be excluded. The total of 26 cases for the current year (2007) is disturbing, possibly symptomatic of the circulation of novel agents. Characterization of these agents should be given a high priority. - Mod.CP]



http://pro-med.blogspot.com/2007/11/proahedr-prion-disease-update-2007-07.html



http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1010,39963




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



http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm



http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf



JOURNAL OF NEUROLOGY

MARCH 26, 2003 RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakobdisease in the United States

Email Terry S. Singeltary:flounder@wt.net

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?



http://www.neurology.org/cgi/eletters/60/2/176#535




THE PATHOLOGICAL PROTEIN

Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9June 2003BY Philip YamCHAPTER 14 LAYING ODDS

Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.



http://www.thepathologicalprotein.com/




Diagnosis and Reporting of Creutzfeldt-Jakob Disease

Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.Terry S. Singeltary, Sr Bacliff, Tex1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323.

FREE FULL TEXT



http://jama.ama-assn.org/cgi/content/extract/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=singeltary&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT



http://jama.ama-assn.org/cgi/content/full/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=singeltary&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT




2 January 2000

British Medical Journal

U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well



http://www.bmj.com/cgi/eletters/320/7226/8/b#6117



15 November 1999

British Medical Journal

vCJD in the USA * BSE in U.S.



http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406





2008

The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.



http://www.cjdfoundation.org/fact.html




CJD TEXAS


http://cjdtexas.blogspot.com/2007/12/creutzfeldt-jakob-disease-surveillance.html



This study further confirms that BASE is caused by a distinct prion isolate and discloses a novel disease phenotype in cattle, closely resembling the phenotype previous reported in scrapie-inoculated cattle

*** and in some subtypes of inherited and sporadic Creutzfeldt-Jakob disease.



http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf



The Italian cases (11 and 15 years of age) originally named bovine amyloidotic spongiform encephalopathy (BASE) were characterized by an unglycosylated protein band with a lower molecular mass (thus named L cases) and the predominance of the monoglycosylated band. In addition, immunohistochemical detection of PrPres in these cases found greater deposits in the cerebral cortex and thalamus versus the brain stem. The French cases found a higher molecular mass associated with the unglycosylated protein band and were called H cases (see figure 1). *** The different "strains" are now called atypical BSE. ...

full text, skroll down to page 6 ;



http://www.usaha.org/committees/reports/2006/report-fe-2006.pdf



MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L, and or Italian L-BASE (the last two cases of mad cow disease in the USA were in Alabama, and Texas, both of which were atypical BSE).



http://bse-atypical.blogspot.com/2008/03/mad-cow-disease-terminology-uk-c-bse.html



Please remember, the last two mad cows documented in the USA i.e. Alabama and Texas, both were of the 'atypical' BSE strain, and immediately after that, the USDA shut down the testing from 470,000 to 40,000 in the U.S. in 2007 out of about 35 million cattle slaughtered. also, science is showing that some of these atypical cases are more virulent to humans than the typical UK BSE strain ;

***Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.***

Progress Report from the National Prion Disease Pathology Surveillance Center
An Update from Stephen M. Sergay, MB, BCh & Pierluigi Gambetti, MD

April 3, 2008



http://www.aan.com/news/?event=read&article_id=4397&page=72.45.45




WHY were they planning to destroy all CJD tissue samples donated ???

Washington Times - Washington,DC,USA NIH may destroy human brain collection
By Steve Mitchell Medical Correspondent

Washington, DC, Mar. 24 (UPI) -- The National Institutes of Health may discard part or all of a rare collection that includes hundreds of human brain samples from patients that suffered from a disorder similar to mad cow disease -- unless another researcher or institution takes them on, United Press International has learned.

Several scientists said the collection, which is held by the NIH's National Institute for Neurological Disorders and Stroke in Bethesda, Md. -- and includes brains and other tissue samples from people afflicted with the brain-wasting illness Creutzfeldt Jakob disease -- is irreplaceable and could even provide insight into treatments for the fatal disorder.

Currently, there is no cure for CJD and patients typically die within a year after symptoms begin.

However, NIH officials in control of the collection's fate told UPI the remaining samples are of little scientific value and may be disposed of if researchers outside the agency do not claim it. That position stands in sharp contrast with CJD experts who thought the collection should be preserved.

"It's invaluable," said Dr. Paul Brown, former medical director of the NIH's Laboratory for Central Nervous System Studies, whose expertise is in CJD and mad cow disease (also known as bovine spongiform encephalopathy, or BSE). ...snip...end...tss



http://www.washtimes.com/upi-breaking/20050323-053919-8481r.htm




NIH says it will preserve CJD brains By STEVE MITCHELL

WASHINGTON, May 31 (UPI) -- The National Institutes of Health apparently has reversed its position on the fate of an invaluable collection of brains from people afflicted with a condition similar to mad cow disease, saying in a letter to a U.S. senator it will not destroy the collection.

snip...

May 10, 2005

The Honorable John Cornyn United States Senator
Occidental Tower5005 LBJ Freeway, Suite 1150 Dallas, Texas 75244-6199

Dear Senator Cornyn:

Your letter to the National Institutes of Health (NIH) forwarding correspondence from Mr. Terry S. Singeltary, Sr., has been forwarded to me for reply. Mr. Singeltary is concerned about the preservation of Creutzfeldt-Jakob disease (CJD) brain samples that have been maintained by the National Institute of Neurological Disorders and Stroke (NINDS) Intramural Research program for many years.

I am sorry to learn that Mr. Singeltary's mother died of CJD and can certainly understand his desire that any tissues that could help investigators unravel the puzzle of this deadly disease are preserved. I hope he will be pleased to learn that all the brains and other tissues with potential to help scientists learn about CJD are, and will continue to be, conserved. (The tissues that are discarded are those that have either decayed to an extent that renders them no longer appropriatefor research or those for which we do not have sufficient identification.) ...snip...end...tss




http://creutzfeldt-jakob-disease.blogspot.com/2008/01/cjd-hgh-body-snatchers.html




NIH says it will preserve CJD brains

Published: May 31, 2005 at 5:26 PM

By STEVE MITCHELL WASHINGTON, May 31 (UPI) -- The National Institutes of Health apparently has reversed its position on the fate of an invaluable collection of brains from people afflicted with a condition similar to mad cow disease, saying in a letter to a U.S. senator it will not destroy the collection.

An NIH official had told United Press International previously that the brain collection, which consists of samples from hundreds of people who died from the brain-wasting illness called Creutzfeldt Jakob disease, could be discarded if another entity does not claim them.

That sparked an outcry from patient-advocacy groups, consumer watchdogs and scientists, and the agency now appears to have backed away from that course.

"All the brains and other tissues with potential to help scientists learn about CJD are, and will continue to be, conserved," Story Landis, director of the National Institute of Neurological Disorders and Stroke, which oversees the brain collection, wrote in a May 10 letter to Sen. John Cornyn, R-Texas.

Cornyn had inquired about the status of the collection in April.

Last March, Eugene Major, acting director of the basic neuroscience program at the NIH, told UPI the useful portions of the collection had been doled out to scientists and the remaining samples had "very little remaining value" and could be destroyed.

Landis could not be reached for comment Tuesday. NINDS spokesman Paul Girolami told UPI he had been unable to locate her.

Scientists think the collection, which dates back to 1963, is invaluable for research on CJD and similar diseases and could even provide insight into treatments. There is no cure for CJD and patients typically die within a year after symptoms begin.

"Absolutely, the collection is worth keeping," Bruce Johnson, a former NIH scientist who said he had been told the collection would be destroyed in two years if no one took the samples from the agency, told UPI.

The Memorial Institute for Neurodegenerative Diseases Inc., a non-profit organization consisting of more than 40 researchers from several countries, offered to take the collection off of NIH's hands more than a year ago and so far has not heard anything from the agency, Harry Peery, MIND's executive director, told UPI.

CJD belongs to a group of incurable and fatal diseases collectively known as transmissible spongiform encephalopathies, or TSEs, that includes mad cow disease in cows, chronic wasting disease in deer and elk, and scrapie in sheep.

Variant CJD, or vCJD, is a relatively new TSE, which people can contract from consuming beef products infected with the mad cow pathogen.

Despite Landis' assurance the collection will be preserved, some family members of the patients who donated their brains to the NIH are still skeptical. This is because the wording Landis used in the letter leaves open the possibility that some brain samples are being destroyed.

"The tissues that are discarded are those that have either decayed to an extent that renders them no longer appropriate for research or those for which we do not have sufficient identification," Landis wrote.

"Which ones" are being destroyed? asked Terry Singeltary, who is involved with several CJD patient groups.

"With a system like this, they could destroy whatever and whenever they wanted, for whatever reason they wanted," Singeltary, whose mother died of CJD in 1997, told UPI.

"It's a perfect excuse to discard some suspicious tissue resembling vCJD or some atypical TSE related to animal TSEs in the USA," he added.

Although the collection includes samples from CJD patients as young as 16 that could make them candidates for possible vCJD, the brains have never been screened for evidence of the disease. The only confirmed vCJD case in the United States occurred in a Florida woman who is thought to have contracted the disease in England.

Johnson said he along with renowned CJD expert Paul Brown were in the process of sorting through the samples to match them up with patient identification documents until they both retired. Some of the samples may prove impossible to identify, he said, but he and Brown are the only ones familiar enough with the collection to organize it and neither has been asked back by the agency to aid in the identification process.
--
Steve Mitchell is UPI's Medical Correspondent. E-mail: sciencemail@upi.com
© 2005 United Press International. All Rights Reserved. This material may not be reproduced, redistributed, or manipulated in any form.



http://www.upi.com/NewsTrack/Science/2005/05/31/nih_says_it_will_preserve_cjd_brains/6771/print_view/




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 nonspecific 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 States will never know the true status of its involvement with BSE.




kind regards,
terry

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