Clinical and Pathological Features and Laboratory Confirmation of Creutzfeldt–Jakob Disease in a Recipient of Pituitary-Derived Human Growth Hormone

Cj, Gibbs; Augustine, Joy; Heffner, Reid R.; Franko, Maryellen C.; Miyazaki, Masao; Dm, Asher; Je, Parisi; Pw, Brown; Dc, Gajdusek

doi:10.1056/nejm198509193131207

Cited by 167 publications

(48 citation statements)

References 17 publications

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“…Younger cases often indicate an environmental source of infection as shown by the unusually young age of initial cases of iatrogenic transmission in the United States and vCJD in the United Kingdom. [17][18][19][20][21][22] Deaths from sporadic prion disease in those aged ,20 years are extremely rare, with only 7 cases of sporadic CreutzfeldtJakob disease previously recorded. 23 In addition to his age, the patient' s multiple concussions were diagnostically deceptive.…”

Section: Discussionmentioning

confidence: 99%

Sporadic Fatal Insomnia in an Adolescent

et al. 2014

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The occurrence of sporadic prion disease among adolescents is extremely rare. A prion disease was confirmed in an adolescent with disease onset at 13 years of age. Genetic, neuropathologic, and biochemical analyses of the patient’s autopsy brain tissue were consistent with sporadic fatal insomnia, a type of sporadic prion disease. There was no evidence of an environmental source of infection, and this patient represents the youngest documented case of sporadic prion disease. Although rare, a prion disease diagnosis should not be discounted in adolescents exhibiting neurologic signs. Brain tissue testing is necessary for disease confirmation and is particularly beneficial in cases with an unusual clinical presentation.

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Section: Discussionmentioning

confidence: 99%

Sporadic Fatal Insomnia in an Adolescent

et al. 2014

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“…), intratongue (i.t. ), subcutaneous, intranasal, and oral routes of exposure (7,9,13,26,27,40,43,51). Peripheral infection is often accelerated by local amplification of agent in follicular dendritic cells (FDC) of lymphoid organs, followed by spread via local nerves to the CNS (12,30,43,47,49,53).…”

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confidence: 99%

Crucial Role for Prion Protein Membrane Anchoring in the Neuroinvasion and Neural Spread of Prion Infection

Klingeborn

Race

Meade-White

et al. 2011

J Virol

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In nature prion diseases are usually transmitted by extracerebral prion infection, but clinical disease results only after invasion of the central nervous system (CNS). Prion protein (PrP), a host-encoded glycosylphosphatidylinositol (GPI)-anchored membrane glycoprotein, is necessary for prion infection and disease. Here, we investigated the role of the anchoring of PrP on prion neuroinvasion by studying various inoculation routes in mice expressing either anchored or anchorless PrP. In control mice with anchored PrP, intracerebral or sciatic nerve inoculation resulted in rapid CNS neuroinvasion and clinical disease (154 to 156 days), and after tongue, ocular, intravenous, or intraperitoneal inoculation, CNS neuroinvasion was only slightly slower (193 to 231 days). In contrast, in anchorless PrP mice, these routes resulted in slow and infrequent CNS neuroinvasion. Only intracerebral inoculation caused brain PrPres, a protease-resistant isoform of PrP, and disease in both types of mice. Thus, anchored PrP was an essential component for the rapid neural spread and CNS neuroinvasion of prion infection.

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“…This method has been fully validated by international bodies (OECD guideline 437, 2009). The BCOP assay was developed as an alternative to the ocular dermal irritation test in rabbits (Draize) (Iccvam, 2003;Gibbs, 1985).…”

Section: Tests For Ocular Irritabilitymentioning

confidence: 99%

Alternative methods in toxicity testing: the current approach

Pucca

Bertolini

Barbosa

et al. 2014

Braz. J. Pharm. Sci.

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Alternative methods are being developed to reduce, refine, and replace (3Rs) animals used in experiments, aimed at protecting animal welfare. The present study reports alternative tests which are based on the principles of the 3Rs and the efforts made to validate these tests. In Europe, several methodologies have already been implemented, such as tests of irritability, cell viability, and phototoxicity as well as in vitro mathematical models together with the use of in silico tools. This is a complex process that spans from development to regulatory approval and subsequent adoption by various official entities. Within this regulatory framework is REACH, the European Community Regulation for chemicals and their safe use. In Brazil, the BraCVAM (Brazilian Center for the Validation of Alternative Methods) was recently established to validate alternative methods and stimulate incorporation of new methodologies. A new vision of toxicology is emerging for the 21st century (Tox-21), and the subsequent changes are shaping a new paradigm.Uniterms: Toxicology/alternative methods. Toxicological analysis/alternative methods. Toxicology/21st. century. Animals/use in experiments.Métodos alternativos estão sendo desenvolvidos para a redução, o refinamento e a substituição (3R) do número de animais utilizados em experimentos, visando ao seu bem-estar. Esses testes alternativos baseiam-se no princípio dos 3R e esforços têm sido empregados para que sejam validados. Na Europa, diversas metodologias já foram implantadas tais como: testes de irritabilidade, testes de viabilidade celular, testes de fototoxicidade e modelos matemáticos in vitro, além do uso de ferramentas in silico. Esse é um processo complexo, que abrange desde o seu desenvolvimento até a aceitação regulatória e posterior adoção por diversas organizações oficiais. No contexto regulatório está o REACH, o Regulamento da Comunidade Européia, para produtos químicos e sua utilização segura. No Brasil, o BraCVAM (Centro Brasileiro de Validação de Métodos Alternativos) foi recentemente estabelecido para validação de métodos alternativos e estímulo à incorporação de novas metodologias. Uma nova visão de toxicologia vem surgindo para o século XXI (Tox-21) e as mudanças ocasionadas promoverão um novo paradigma.Unitermos: Toxicologia/métodos alternativos. Análise toxicológica/métodos alternativos. Toxicologia/ século 21. Animais/uso em experimentos.

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Clinical and Pathological Features and Laboratory Confirmation of Creutzfeldt–Jakob Disease in a Recipient of Pituitary-Derived Human Growth Hormone

Cited by 167 publications

References 17 publications

Sporadic Fatal Insomnia in an Adolescent

Sporadic Fatal Insomnia in an Adolescent

Crucial Role for Prion Protein Membrane Anchoring in the Neuroinvasion and Neural Spread of Prion Infection

Alternative methods in toxicity testing: the current approach

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