CWD TSE Prion in cervids to hTGmice, Heidenhain Variant Creutzfeldt-Jacob
Disease MM1 genotype, and iatrogenic CJD ???
P07 Behavioral Neurology: Aging and Dementia MRI More Useful Than PET for
Diagnosis of Heidenhain Variant Creutzfeldt-Jacob Disease (P07.163)
Jonathan Beary1 and Edward Manno2 1 General Neurology, Neurological
Institute Cleveland Clinic Cleveland OH 2 Cerebrovascular Neurology,
Neurological Institute Cleveland Clinic Cleveland OH
OBJECTIVE: To demonstrate that MRI detection of subtle focal cortical
abnormalities can prove more useful than positron emission tomography (PET) in
the diagnosis of Heidenhain variant Creutzfeldt-Jakob Disease (hvCJD).
BACKGROUND: hvCJD is a rare neurodegenerative, spongiform encephalopathy
with an aggressive clinical course. PET brain imaging has been reported to
detect focal cortical abnormalities in hvCJD with greater sensitivity than MRI.
However, because PET is both more costly and less accessable than MRI, early
diagnosis of this disease and subsequent prognostication may be unnecessarily
delayed. The reliability of MRI over PET in detecting isolated occipital
cortical changes suggestive of hvCJD has not been well studied.
DESIGN/METHODS: This is a case report with relevent neuroimaging review.
RESULTS: A 70 year-old right-handed male experienced visual hallucinations
and visuospatial disorientation with worsening ataxia followed by progressive
anterograde amnesia and cortical blindness. Six weeks later he was comatose with
startle myoclonus. A sharply-contoured periodic pattern was evident posteriorly
on continuous EEG monitoring with brain MRI revealing subtle bilateral occipital
cortical diffusion restriction. PET brain imaging showed diffuse non-focal
cortical hypometabolism. Both cerebrospinal fluid (CSF) 14-3-3 and tau protein
studies were positive. EEG progressed to refractory status epilepticus and the
patient died four days later. ***The presence of abnormal brain
protease-resistant prion protein and MM1 genotype at autopsy supported the
diagnosis of hvCJD.
CONCLUSIONS: hvCJD should be considered in patients with rapid-onset
idiopathic visual disturbance and dementia. When combined with EEG and CSF
analysis, isolated MRI visual cortex diffusion restriction is suggestive of this
ultra-aggressive prion variant. MRI is able to efficiently facilitate valuable
prognostication early in hvCJD and can be more useful than costly PET imaging.
Disclosure: Dr. Beary has nothing to disclose. Dr. Manno has nothing to
disclose.
> The presence of abnormal brain protease-resistant prion protein and
MM1 genotype at autopsy supported the diagnosis of hvCJD.
Wednesday, January 01, 2014
Molecular Barriers to Zoonotic Transmission of Prions
*** chronic wasting disease, there was no absolute barrier to conversion of
the human prion protein.
*** Furthermore, the form of human PrPres produced in this in vitro assay
when seeded with CWD, resembles that found in the most common human prion
disease, namely sCJD of the MM1 subtype.
Subtype 1: (sCJDMM1 and sCJDMV1)
This subtype is observed in patients who are MM homozygous or MV
heterozygous at codon 129 of the PrP gene (PRNP) and carry PrPSc Type 1.
Clinical duration is short, 3‑4 months.32 The most common presentation in
sCJDMM1 patients is cognitive impairment leading to frank dementia, gait or limb
ataxia, myoclonic jerks and visual signs leading to cortical blindness
(Heidenhain’s syndrome)...
Animals injected with iatrogenic Creutzfeldt–Jakob disease MM1 and genetic
Creutzfeldt–Jakob disease MM1 linked to the E200K mutation showed the same
phenotypic features as those infected with sporadic Creutzfeldt–Jakob disease
MM1 prions...
*** our results raise the possibility that CJD cases classified as VV1 may
include cases caused by iatrogenic transmission of sCJD-MM1 prions or food-borne
infection by type 1 prions from animals, e.g., chronic wasting disease prions in
cervid. In fact, two CJD-VV1 patients who hunted deer or consumed venison have
been reported (40, 41). The results of the present study emphasize the need for
traceback studies and careful re-examination of the biochemical properties of
sCJD-VV1 prions. ***
snip...see full text ;
Wednesday, June 16, 2010
Defining sporadic Creutzfeldt-Jakob disease strains and their transmission
properties
The epidemiological findings in sCJD demonstrate that approximately 80% of
patients are diagnosed with “classic CJD” types MM1 and MV1, which might
intriguingly suggest an infectious rather than genetic origin for the majority
of sCJD cases.
snip...
Therefore if sCJD(MV2) and sCJD(VV2) were to become iatrogenic sources of
human infection, the host response may be indistinguishable from sCJD(MM1) and
more transmissible with respect to further infection.
END...TSS
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
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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.
snip...see full text ;
Wednesday, January 01, 2014
APHIS-2006-0118-0100 Chronic Wasting Disease Herd Certification Program and
Interstate Movement of Farmed or Captive Deer, Elk, and Moose
Monday, December 02, 2013
*** A parliamentary inquiry has been launched today into the safety of
blood, tissue and organ screening following fears that vCJD – the human form of
‘mad cow’ disease – may be being spread by medical procedures
Wednesday, December 11, 2013
Detection of Infectivity in Blood of Persons with Variant and Sporadic
Creutzfeldt-Jakob Disease
Friday, November 22, 2013
Chronic Wasting disease CWD is threat to the entire UK deer population
Singeltary submission to the Scottish Parliament
Terry S. Singeltary Sr., MOM DOD 12/14/97 confirmed Heidenhain Variant of
Creutzfeldt Jakob Disease hvCJD...just made a promise to her, never forget, and
never let them forget. ...
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