Neuropathology of scrapie: a study of the distribution patterns of brain lesions in 222 cases of natural scrapie in sheep, 1982–1991

Wood, JL; McGill, Iain; Done, S.H.; Bradley, Ray

doi:10.1136/vr.140.7.167

Cited by 77 publications

(60 citation statements)

References 33 publications

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“…The distribution of histopathologic lesions within the CNS described in these naturally infected goats is generally in agreement with what has been described for naturally scrapie-affected sheep. 36 In particular, the DMNV at the level of the obex in the medulla oblongata was consistently affected. Widespread PrP sc deposition in the CNS and peripheral lymphoid tissues of a single naturally infected clinical scrapie goat was reported from the USA using IHC.…”

Section: Discussionmentioning

confidence: 97%

Atypical Scrapie in a Swiss Goat and Implications for Transmissible Spongiform Encephalopathy Surveillance

et al. 2007

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Abstract. Different types of transmissible spongiform encephalopathies (TSEs) affect sheep and goats. In addition to the classical form of scrapie, both species are susceptible to experimental infections with the bovine spongiform encephalopathy (BSE) agent, and in recent years atypical scrapie cases have been reported in sheep from different European countries. Atypical scrapie in sheep is characterized by distinct histopathologic lesions and molecular characteristics of the abnormal scrapie prion protein (PrP sc ). Characteristics of atypical scrapie have not yet been described in detail in goats. A goat presenting features of atypical scrapie was identified in Switzerland. Although there was no difference between the molecular characteristics of PrP sc in this animal and those of atypical scrapie in sheep, differences in the distribution of histopathologic lesions and PrP sc deposition were observed. In particular the cerebellar cortex, a major site of PrP sc deposition in atypical scrapie in sheep, was found to be virtually unaffected in this goat. In contrast, severe lesions and PrP sc deposition were detected in more rostral brain structures, such as thalamus and midbrain. Two TSE screening tests and PrP sc immunohistochemistry were either negative or barely positive when applied to cerebellum and obex tissues, the target samples for TSE surveillance in sheep and goats. These findings suggest that such cases may have been missed in the past and could be overlooked in the future if sampling and testing procedures are not adapted. The epidemiological and veterinary public health implications of these atypical cases, however, are not yet known.

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Section: Discussionmentioning

confidence: 97%

Atypical Scrapie in a Swiss Goat and Implications for Transmissible Spongiform Encephalopathy Surveillance

et al. 2007

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“…1,10 In the past, classic scrapie disease was confirmed by examination of the brain tissue for a triad of salient histopathologic changes, including vacuolation limited to gray matter referred to as spongiform change, loss of neurons, and accompanying gliosis. 26 However, more recently, a presumptive histopathologic diagnosis was largely replaced by the more sensitive and confirmatory IHC and biochemical methods for detection of PrP Sc in the brain or lymphoid tissues. In the case of Nor98 scrapie, there is generally little or no vacuolation in the brain, and, to date, no lymphoid accumulation of PrP Sc has been detected.…”

Section: Discussionmentioning

confidence: 99%

Nor98 Scrapie Identified in the United States

et al. 2009

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Abstract. A distinct strain of scrapie identified in sheep of Norway in 1998 has since been identified in numerous countries throughout Europe. The disease is known as Nor98 or Nor98-like scrapie, among other names. Distinctions between classic scrapie and Nor98 scrapie are made based on histopathology and immunodiagnostic results. There are also differences in the epidemiology, typical signalment, and likelihood of clinical signs being observed. In addition, sheep that have genotypes associated with resistance to classic scrapie are not spared from Nor98 disease. The various differences between classic and Nor98 scrapie have been consistently reported in the vast majority of cases described across Europe. The current study describes in detail the pathologic changes and diagnostic results of the first 6 cases of Nor98 scrapie disease diagnosed in sheep of the United States.

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“…2,5 In domestic animals, P. insidiosum is best known as a cutaneous pathogen of horses, 1 and a gastrointestinal or cutaneous pathogen of dogs. 5 It has also been described as a rare cause of cutaneous disease in cats 20 and calves 18 , intestinal disease in horses, 17 and arteritis, keratitis, or periorbital cellulitis in humans. 19 The infective form of P. insidiosum is thought to be the motile biflagellate zoospore, which is released into aquatic environments and likely causes infection by encysting in damaged skin, gastrointestinal mucosa, or cornea.…”

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confidence: 99%

“…2 The term typical scrapie is used in this report to refer to the disease as defined before Nor98 was identified. 13,15,20 The active surveillance for TSEs in sheep in Sweden in 2003 resulted in identification of 4 cases of Nor98. 13,15,20 The purpose of this report is to describe the presentation of this newly recognized variant of scrapie of sheep and to document its occurrence outside Norway.…”

mentioning

confidence: 99%

“…13,15,20 The active surveillance for TSEs in sheep in Sweden in 2003 resulted in identification of 4 cases of Nor98. 13,15,20 The purpose of this report is to describe the presentation of this newly recognized variant of scrapie of sheep and to document its occurrence outside Norway. The age, breed, and genotype of the ewes and results of the ELISA, IHC, and WB of the 4 cases are presented in Table 1.…”

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confidence: 99%

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Recognition of the Nor98 Variant of Scrapie in the Swedish Sheep Population

et al. 2004

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Abstract. Within the framework of the active surveillance for transmissible spongiform encephalopathies in sheep in Sweden, 4 cases of the atypical form of scrapie, Nor98, were identified during 2003. Nor98 is a recently recognized and poorly understood variant of scrapie, first described in Norway. The cases were positive by the rapid test (enzyme-linked immunosorbent assay). Immunohistochemical staining showed diffuse thingranular staining of the cerebellar cortex. Western immunoblotting analysis of specimens of brain stem and cerebellum showed a light band of approximately 12 kDa. Typical scrapie was ruled out based on the confirmatory testing. The affected ewes were from 4 different flocks. They were between 7 and 9 years old. Two were of the ARQ/ARQ genotype, 1 ARR/ARQ, and 1 ARR/AHQ. Two ewes had shown ataxia, and the other 2 had no clinical signs. Whole-flock slaughter was applied, and testing of the flock mates did not reveal additional cases. Nor98 differs from typical scrapie in its epidemiology, frequency of genotypes of sheep affected, clinical signs, microscopic lesions, distribution of scrapie prion protein in the brain, and characteristics of the immunostaining and immunoblotting profiles.

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Neuropathology of scrapie: a study of the distribution patterns of brain lesions in 222 cases of natural scrapie in sheep, 1982–1991

Cited by 77 publications

References 33 publications

Atypical Scrapie in a Swiss Goat and Implications for Transmissible Spongiform Encephalopathy Surveillance

Atypical Scrapie in a Swiss Goat and Implications for Transmissible Spongiform Encephalopathy Surveillance

Nor98 Scrapie Identified in the United States

Recognition of the Nor98 Variant of Scrapie in the Swedish Sheep Population

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