Research article
Pruritus is a common feature in sheep infected with the BSE agent
Timm Konold , Gemma Bone , Alberto Vidal-Diez , Raul Tortosa , Andrew Davis , Glenda Dexter , Peter Hill , Martin Jeffrey , Marion M Simmons , Melanie J Chaplin , Susan J Bellworthy and Christine Berthelin-Baker
BMC Veterinary Research 2008, 4:16doi:10.1186/1746-6148-4-16
Published: 29 April 2008
Abstract (provisional)
Background
The variability in the clinical or pathological presentation of transmissible spongiform encephalopathies (TSEs) in sheep, such as scrapie and bovine spongiform encephalopathy (BSE), has been attributed to prion protein genotype, strain, breed, clinical duration, dose, route and type of inoculum and the age at infection. The study aimed to describe the clinical signs in sheep infected with the BSE agent throughout its clinical course to determine whether the clinical signs were as variable as described for classical scrapie in sheep. The clinical signs were compared to BSE-negative sheep to assess if disease-specific clinical markers exist.
Results Forty-seven (34%) of 139 sheep, which comprised 123 challenged sheep and 16 undosed controls, were positive for BSE. Affected sheep belonged to five different breeds and three different genotypes (ARQ/ARQ, VRQ/VRQ and AHQ/AHQ). None of the controls or BSE exposed sheep with ARR alleles were positive. Pruritus was present in 41 (87%) BSE positive sheep; the remaining six were judged to be pre-clinically infected. Testing of the response to scratching along the dorsum of a sheep proved to be a good indicator of clinical disease with a test sensitivity of 85% and specificity of 98% and usually coincided with weight loss. Clinical signs that were displayed significantly earlier in BSE positive cases compared to negative cases were behavioural changes, pruritic behaviour, a positive scratch test, alopecia, skin lesions, teeth grinding, tremor, ataxia, loss of weight and loss of body condition. The frequency and severity of each specific clinical sign usually increased with the progression of disease over a period of 16-20 weeks.
Conclusions Our results suggest that BSE in sheep presents with relatively uniform clinical signs, with pruritus of increased severity and abnormalities in behaviour or movement as the disease progressed. Based on the studied sheep, these clinical features appear to be independent of breed, affected genotype, dose, route of inoculation and whether BSE was passed into sheep from cattle or from other sheep, suggesting that the clinical phenotype of BSE is influenced by the TSE strain more than by other factors. The clinical phenotype of BSE in the genotypes and breed studied was indistinguishable from that described for classical scrapie cases.
http://www.biomedcentral.com/1746-6148/4/16/abstract
FULL TEXT *** ;
http://www.biomedcentral.com/content/pdf/1746-6148-4-16.pdf
Harash Narang
Like all other common infections, over twenty different strains of the scrapie agent has been identified from sheep. A search of the literature indicates two distinct clinical syndromes in sheep, both of which have been called scrapie.
I have designated these, Type I (the common type), which exhibit itchiness and lose their wool, and Type II, which exhibit trembling and ataxia.
http://www.cjdfoundation.com/harash.htm
http://lib.bioinfo.pl/auth:Narang,HK
http://www.bseinquiry.gov.uk/files/ws/s113.pdf
An Edmonton company is confident it has a cheap, ground-breaking test for mad cow disease, but the test's British inventor who claims to have first made the link between BSE and the disease's human form insists he still holds the rights. Despite their differences, inventor Harash Narang and BSE Prion Solutions Inc. agree the test holds amazing potential to quickly and inexpensively test live cattle for bovine spongiform encephalopathy better known as mad-cow disease. He is no longer part of the research, but work on it continues at the United States National Prion Disease Pathology Surveillance Center at Case Western Reserve University in Cleveland.
http://blog.case.edu/casedaily/2006/07/21/case_daily
NEUROLOGY 1996;46:940-941 © 1996 American Academy of Neurology
Pruritus in Creutzfeldt-Jakob disease H. Shabtai, P. Nisipeanu, J. Chapman and Korczyn A.D. From the Sackler Faculty of Medicine, Tel Aviv University. Tel Aviv, Israel. Received May 3, 1995. Accepted in final form September 19. 1995. Address correspondence and reprint requests to Professor A.D. Korczyn, Sackler Faculty of Medicine, Tel Aviv University, Ramat Aviv, 69978, Israel.
We report three patients with Creutzfeldt-Jakob disease, whose first symptom was severe pruritus, similar to that observed in scrapie. The pruritus was resistant to therapy. The underlying mechanisms are unclear, but we speculate that the pruritus may result from brainstem involvement.
NEUROLOGY 1996;46: 940-941.
http://www.neurology.org/cgi/content/abstract/46/4/940
Journal of Neurology, Neurosurgery, and Psychiatry, 1993, Vol 56, 1109-1112
--------------------------------------------------------------------------------
PAPERS
Clinical heterogeneity and unusual presentations of Creutzfeldt-Jakob disease in Jewish patients with the PRNP codon 200 mutation J Chapman, P Brown, LG Goldfarb, A Arlazoroff, DC Gajdusek and AD Korczyn Department of Neurology, Tel-Aviv Medical Center, Ichilov Hospital, Israel.
The cluster of Creutzfeldt-Jakob disease among Jews of Libyan origin is the largest in the world. It was found that the disease in this ethnic group is linked to a point mutation in codon 200 of the prion protein precursor gene. In this study the clinical data from 14 such patients are described, demonstrating wide phenotypic heterogeneity. The age of onset ranged from 34 to 65 years and the duration of disease from 2 to 66 months. Clinical features included cerebral, basal ganglia, brainstem, cerebellar, and spinal cord dysfunction. Uncommon features included fatal insomnia in one patient, pruritus in another, and demyelinating peripheral neuropathy in two.
--------------------------------------------------------------------------------
© 1993 by Journal of Neurology, Neurosurgery, and Psychiatry
http://jnnp.bmj.com/cgi/content/abstract/56/10/1109
see more ;
http://www.google.com/search?hl=en&q=creutzfeldt+jakob+disease+Pruritus&btnG=Search
Thursday, April 24, 2008
RE-FOIA OF DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E. OF FOREIGN ORIGIN IN THE UNITED STATES [Docket No. 00-072-1]
http://foiamadsheepmadrivervalley.blogspot.com/2008/04/re-foia-of-declaration-of-extraordinary.html
NOR-98 ATYPICAL SCRAPIE USA UPDATE AS AT OCT 2007
http://nor-98.blogspot.com/
Sunday, April 20, 2008
Progress Report from the National Prion Disease Pathology Surveillance Center April 3, 2008
http://prionunitusaupdate2008.blogspot.com/
TSS