Recently described scrapie-like encephalopathies of animals: case definitions

Wells, G. A. H.; McGill, Iain

doi:10.1016/0034-5288(92)90076-e

Cited by 66 publications

(43 citation statements)

References 52 publications

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“…3,6,7,15,30,33,36,42,50,55,58,60,63,68,70 Based initially on spongiform changes and examination of large case numbers, the invariable predominance of lesions in the brainstem established this and, specifically, the medulla oblongata as the predilection brain region for diagnostic sampling. 18,30,53,63,66,68,70 Correlation of the highest concentrations of PrP Sc in the brainstem with the greatest degree of spongiform change 30,43,51,68 is considered suggestive of the initial brain pathologic alternations in BSE occurring in this region, consistent with the proposed pathogenesis of transmissible spongiform encephalopathy (TSE) in ruminants and laboratory rodent models after oral exposure. 4,5,13,20,26,29,39,40,61 This is supported by observations in a subset of clinical cases with restricted PrP Sc accumulation, which was invariably confined to the brainstem.…”

Section: Of Tissue Accumulations Of Prpmentioning

confidence: 76%

Experimental Classical Bovine Spongiform Encephalopathy

et al. 2010

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Tissues from sequential-kill time course studies of bovine spongiform encephalopathy (BSE) were examined to define PrP immunohistochemical labeling forms and map disease-specific labeling over the disease course after oral exposure to the BSE agent at two dose levels. Study was confined to brainstem, spinal cord, and certain peripheral nervous system ganglia-tissues implicated in pathogenesis and diagnosis or disease control strategies. Disease-specific labeling in the brainstem in 39 of 220 test animals showed the forms and patterns observed in natural disease and invariably preceded spongiform changes. A precise temporal pattern of increase in labeling was not apparent, but labeling was generally most widespread in clinical cases, and it always involved neuroanatomic locations in the medulla oblongata. In two cases, sparse labeling was confined to one or more neuroanatomic nuclei of the medulla oblongata. When involved, the spinal cord was affected at all levels, providing no indication of temporal spread within the cord axis or relative to the brainstem. Where minimal PrP labeling occurred in the thoracic spinal cord, it was consistent with initial involvement of general visceral efferent neurons. Labeling of ganglia involved only sensory ganglia and only when PrP was present in the brainstem and spinal cord. These experimental transmissions mimicked the neuropathologic findings in BSE-C field cases, independent of dose of agent or stage of disease. The model supports current diagnostic sampling approaches and control measures for the removal and destruction of nervous system tissues in slaughtered cattle.

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Section: Of Tissue Accumulations Of Prpmentioning

confidence: 76%

Experimental Classical Bovine Spongiform Encephalopathy

et al. 2010

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“…BSE is transmissible via BSE-contaminated feed to felines (feline spongiform encephalopathy, FSE) and exotic ungulates (exotic ungulate encephalopathy, EUE). 50,51 Prions are proteinaceous infectious particles and are the causative agents of TSEs. They are host-coded proteins that have undergone conformational changes and have biological and physicochemical characteristics that differ significantly from those of other infectious agents.…”

Section: Introductionmentioning

confidence: 99%

Identification and Characterization of two Bovine Spongiform Encephalopathy cases Diagnosed in the United States

et al. 2007

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Abstract. Bovine spongiform encephalopathy (BSE) is a transmissible spongiform encephalopathy of cattle, first detected in 1986 in the United Kingdom and subsequently in other countries. It is the most likely cause of variant Creutzfeldt-Jakob disease (vCJD) Sc from case 1 showed molecular features similar to typical BSE isolates, whereas PrP Sc from case 2 revealed an unusual molecular PrP Sc pattern: molecular mass of the unglycosylated and monoglycosylated isoform was higher than that of typical BSE isolates and case 2 was strongly labeled with antibody P4, which is consistent with a higher molecular mass. Sequencing of the prion protein gene of both BSE-positive animals revealed that the sequences of both animals were within the range of the prion protein gene sequence diversity previously reported for cattle.

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“…Ruminants are the major group of mammals affected by TSEs, with scrapie in sheep and goats and bovine spongiform encephalopathy (BSE) in cattle. Similar diseases have now been recognised also in a number of captive exotic ruminants (Wells and McGill 1991). Until recently, mink was the only carnivore reported to be affected by a naturally occurring spongiform encephalopathy, transmissible mink encephalopathy (TME) (Hartsough and Burger 1965).…”

Section: Introductionmentioning

confidence: 87%

“…The modified PrP product is amyloid in nature and is deposited in the form of fibrils, the scrapie-associated fibrils, or immunochemically in nervous tissue extracts (Kimberlin 1990). Histological changes in the brain of neuropil vacuolation, vacuolation of neuronal perikarya and an astrocytic reaction are characteristic of TSE (Wells and McGill 1991).…”

Section: Transmissible Spongiform Encephalopathiesmentioning

confidence: 99%