Two Irish cases of scrapie resembling Nor98

Onnasch, H.; Gunn, H. M.; Bradshaw, Bernard; Benestad, Sylvie L.; Bassett, Hugh F.

doi:10.1136/vr.155.20.636

Cited by 79 publications

(67 citation statements)

References 11 publications

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“…Hind limb ataxia was also reported in two Swedish cases, one of them was also described as stiff and with poor body condition, while other Swedish cases had not displayed any clinical symptoms according to the farmer [16]. Two clinical suspect cases reported in Ireland shared the same clinical sign, incoordination, in addition to weight loss and nervous behaviour [32]. The case in the Falkland Islands displayed poor body condition and an abnormal behaviour with persistent biting at its leg and had shown collapsing episodes when it tried to run [14].…”

Section: Clinical Signs In Atypical/nor98 Scrapie Sheepmentioning

confidence: 73%

“…But in these areas too, small differences are noticeable. A diffuse fine punctate or alternatively a patchy staining (never stellate), in some cases very discrete while conspicuous in other cases, is reported in the molecular and granular layers and in the white matter, or in two or even in only one of these three structures [5,9,10,16,31,32,37]. It is important to underline that immunostaining can be minimal or totally absent from the cerebellum in some atypical/Nor98 scrapie cases while in other cases the only brain area showing immunostaining is in the cerebellum 3 [31].…”

Section: Atypical/nor98 Scrapie Brains Have a Different Appearance Anmentioning

confidence: 99%

“…In the brainstem, some cases have been described as totally negative at the level of the obex [5,9,10,16,32] while other cases display minimal staining restricted only to the spinal tract nucleus of the trigeminal nerve [5,31,33,37,41]. The primary structure affected in classical scrapie, the dorsal motor nucleus of the vagus (DMNV) was never affected.…”

Section: Atypical/nor98 Scrapie Brains Have a Different Appearance Anmentioning

confidence: 99%

“…Second, the results of the tests depend on which part of the brain the test is applied to since the amount of PrP Sc is different in atypical/Nor98 scrapie cases compared to classical scrapie [16]. Third, the choice of the test for confirmation and the brain region investigated for confirmation will also affect the ability to detect atypical scrapie, since it has been reported that the atypical/Nor98 scrapie cases might be negative by immunohistochemistry at the level of the obex [5,9,10,16,32]. And then finally, the population structure (age-structure, predominant genotypes, systems for collecting fallen stock) and the design of the surveillance programme can also have an effect on the detected prevalence.…”

Section: Prevalence and Factors Affecting The Ability To Detect Atypimentioning

confidence: 99%

“…The vast majority of these atypical cases are similar if not identical to Nor98 cases, but they are designated outside Norway as "Nor98", "Nor98-like", or "atypical scrapie". The authors therefore choose to the majority of the European countries [9,13,15,16,[31][32][33]37], with also one case in the Falkland Islands [14] and in the USA [12]. A case was recently retrospectively found in UK archive material dating from 1989 [7].…”

Section: Introductionmentioning

confidence: 99%

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Atypical/Nor98 scrapie: properties of the agent, genetics, and epidemiology

et al. 2008

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-Atypical/Nor98 scrapie cases in sheep were diagnosed for the first time in Norway in 1998. They are now identified in small ruminants in most European countries and represent an increasingly large proportion of the scrapie cases diagnosed in Europe. Atypical/Nor98 scrapie isolates have shown to be experimentally transmissible into transgenic mice and sheep but the properties of the TSE agent involved, like its biological and biochemical features, are so clearly distinct from the agent involved in classical scrapie that they have provided a challenging diagnostic for many years. No strain diversity has yet been identified among the atypical/Nor98 scrapie sample cases. The genetic predisposition of the sheep affected by atypical/Nor98 scrapie is almost inverted compared to classical scrapie, and the exact origin of this sporadic TSE strain is still speculative, but a spontaneous, non-contagious origin, like sporadic CreutzfeldtJakob disease in humans, can not be excluded. Further transmission and epidemiological studies are needed to better address this hypothesis. atypical scrapie / Nor98 / transmissible spongiform encephalopathies TSE / genetics / epidemiology

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Section: Clinical Signs In Atypical/nor98 Scrapie Sheepmentioning

confidence: 73%

Section: Atypical/nor98 Scrapie Brains Have a Different Appearance Anmentioning

confidence: 99%

Section: Atypical/nor98 Scrapie Brains Have a Different Appearance Anmentioning

confidence: 99%

Section: Prevalence and Factors Affecting The Ability To Detect Atypimentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Atypical/Nor98 scrapie: properties of the agent, genetics, and epidemiology

et al. 2008

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2006

EFSA Journal

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Atypical Prion Diseases in Humans and Animals

Tranulis

Benestad

Baron

et al. 2011

Topics in Current Chemistry

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Although prion diseases, such as Creutzfeldt-Jakob disease (CJD) in humans and scrapie in sheep, have long been recognized, our understanding of their epidemiology and pathogenesis is still in its early stages. Progress is hampered by the lengthy incubation periods and the lack of effective ways of monitoring and characterizing these agents. Protease-resistant conformers of the prion protein (PrP), known as the "scrapie form" (PrP(Sc)), are used as disease markers, and for taxonomic purposes, in correlation with clinical, pathological, and genetic data. In humans, prion diseases can arise sporadically (sCJD) or genetically (gCJD and others), caused by mutations in the PrP-gene (PRNP), or as a foodborne infection, with the agent of bovine spongiform encephalopathy (BSE) causing variant CJD (vCJD). Person-to-person spread of human prion disease has only been known to occur following cannibalism (kuru disease in Papua New Guinea) or through medical or surgical treatment (iatrogenic CJD, iCJD). In contrast, scrapie in small ruminants and chronic wasting disease (CWD) in cervids behave as infectious diseases within these species. Recently, however, so-called atypical forms of prion diseases have been discovered in sheep (atypical/Nor98 scrapie) and in cattle, BSE-H and BSE-L. These maladies resemble sporadic or genetic human prion diseases and might be their animal equivalents. This hypothesis also raises the significant public health question of possible epidemiological links between these diseases and their counterparts in humans.

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Two Irish cases of scrapie resembling Nor98

Cited by 79 publications

References 11 publications

Atypical/Nor98 scrapie: properties of the agent, genetics, and epidemiology

Atypical/Nor98 scrapie: properties of the agent, genetics, and epidemiology

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Atypical Prion Diseases in Humans and Animals

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