Sunday, May 03, 2015

Unusual Case of Sporadic Creutzfeldt–Jakob Disease Subtype VV1

Unusual Case of Sporadic Creutzfeldt–Jakob Disease Subtype VV1

 

 

To the Editor: Creutzfeldt-Jakob disease is characterized by a variety of symptoms, including rapidly progressive dementia, ataxia, and myoclonus.

 

Sporadic Creutzfeldt-Jakob disease (sCJD) has a median disease duration of 6 months.1

 

Seven different molecular subtypes of sCJD hae been identified (MM1, MM2-C, MM2T, MV1, MV2, VV1, and VV2), based on prion protein gene (PRNP) codon 129 polymorphism and the prion protein type 2.

 

VV1 is the rarest molecular subtype of sCJD (only about 1% of cases). To date, reported cases of sCJD VV1 have been characterized by young age at disease onset, long duration, and progressive dementia.

 

We describe a unique case of sCJD VV1 with older age at disease onset and shorter illness duration.

 
 

CASE REPORT

 

snip...

 
 
 


Free first page
 
 
 
 
 

Wednesday, April 08, 2015

Sporadic Creutzfeldt-Jakob Disease MM1+2C and MM1 Are Identical in Transmission Properties
http://creutzfeldt-jakob-disease.blogspot.com/2015/04/sporadic-creutzfeldt-jakob-disease.html
 
Saturday, April 04, 2009
 
An unusually presenting case of sCJD—The VV1 subtype Volume 111, Issue 3, Pages 282-291 (April 2009)
 
An unusually presenting case of sCJD—The VV1 subtype
 
Kaloyan S. Taneva, Mimi Yilmab
 
Received 16 November 2007; received in revised form 4 September 2008; accepted 12 September 2008.
 
Abstract
 
Creutzfeldt–Jakob disease (CJD) is a rapidly progressive neurodegenerative disease caused by prions. Typically CJD presents with a triad of rapidly progressive dementia, abnormal movements (e.g., myoclonus) and electroencephalographic (EEG) changes. Recently, CJD has been subdivided into subtypes based on host genetic polymorphisms and the characteristics of the pathological prion protein. Different subtypes likely have different clinical and laboratory presentations. We describe a case of sporadic CJD of the VV1 subtype. We describe our patient's clinical symptoms, time course, laboratory workup, structural and functional neuroimaging data, EEG data and CJD biomarkers. Our patient presented with clinical symptoms atypical for CJD. Because of that, her clinical symptoms were initially attributed to psychiatric reasons. After extensive clinical and laboratory investigation, we concluded that the patient probably had CJD. Postmortem neuropathological results confirmed this clinical hypothesis. We compare our patient's clinical, laboratory and neuroimaging data to the data on typical CJD as well as the data on the few CJD VV1 cases described in the literature. We discuss our case's relevance to the diagnosis of CJD.
 
Keywords: Creutzfeldt–Jakob disease, Dementia, Neuroimaging, Magnetic resonance imaging, Electroencephalography, Biomarkers, Prion diseases a Department of Psychiatry, Massachusetts General Hospital, 55 Fruit Street, Warren 1220/Blake 11, Boston, MA 02114, United States
 
b University of Connecticut Health Center, Farmington, CT, United States
 
Corresponding author. Tel.: +1 617 726 7511; fax: +1 617 724 9155.
 
PII: S0303-8467(08)00320-X
 
doi:10.1016/j.clineuro.2008.09.017
 
© 2008 Elsevier B.V. All rights reserved.
 
 
 
rare atypical strain of sporadic cjd ??? seems these rare strains are increasing ???
 
Wednesday, February 04, 2009
 
Creutzfeldt-Jacob disease presenting as severe depression: a case report
 
 
 
A case-control study of sporadic Creutzfeldt-Jakob disease in Switzerland: analysis of potential risk factors with regard to an increased CJD incidence in the years 2001-2004
 
 
 
Thursday, July 10, 2008
 
A New Prionopathy update July 10, 2008
 
snip...
 
DOES ANYONE BESIDES ME SEE A PATTERN YET ???
 
Vickey Rimmer, 16, DID NOT DIE FROM nvCJD, she died from a form of sporadic CJD, whatever the hell that is. and there have been 16 year old die from sporadic CJD in the USA as well.
 
SIMPLY PUT, the ukbsenvcjd only theory was wrong from day one. the elderly are expendable, pets and kids are not.
 
Science was dictated by 'big buisness' after the Vickey Rimmer case with the ukbsenvcjd only myth.
 
snip...
 
Sporadic creutzfeldt-jakob disease in two adolescents
 
 
 
see full text sporadic CJD the big lie;
 
snip...
 
IT seems we have come full circle from the 'ORIGINAL 10' i.e. the 1st 10 adolescents in the UKBSEnvCJD only theory. and now we find us at the 1st 10 in USA, or is it the first 10, or the tip of the iceburg, many that went undocumented ???
 
lets look at the full circle, to date ;
 
 
 
Sunday, August 10, 2008
 
A New Prionopathy OR more of the same old BSe and sporadic CJD
 
 
 
HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory JUNE 2008
 
snip...
 
Tissue infectivity and strain typing of the many variants Manuscript 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. ...
 
snip...
 
 
 
Elsevier Editorial System(tm) for The Lancet Infectious Diseases Manuscript Draft Manuscript Number: Title: HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory Article Type: Personal View Corresponding Author: Mr. Terry S. Singeltary, Corresponding Author's Institution: na First Author: Terry S Singeltary, none Order of Authors: Terry S Singeltary, none; Terry S. Singeltary
 
Abstract:
 
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. snip...
 
see full text 31 pages ;
 
 
 
Friday, November 30, 2007
 
CJD QUESTIONNAIRE USA CWRU AND CJD FOUNDATION
 
 
 
Saturday, April 04, 2009
 
An unusually presenting case of sCJD—The VV1 subtype
 
 
 
Tuesday, April 21, 2015
 
Transmissible Spongiform Encephalopathy Advisory Committee TSEAC MEETING SCHEDULED FOR June 1, 2015
 
 


 
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