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Order Sepolyvirales
Published in Paul Pumpens, Peter Pushko, Philippe Le Mercier, Virus-Like Particles, 2022
Paul Pumpens, Peter Pushko, Philippe Le Mercier
Developing further the pseudotyped HaPyV VLP approach, Eiden et al. (2021) engineered a prospective vaccine against bovine spongiform encephalopathy (BSE) in cattle, a disease like scrapie in sheep or Creutzfeldt-Jakob disease in humans that are fatal neurodegenerative diseases characterized by the conformational conversion of the normal, mainly α-helical cellular prion protein (PrPC) into the abnormal β-sheet rich infectious isoform PrPSc. Since the immunization against prions is hampered by the self-tolerance to PrPC in mammalian species, the authors presented the nine different PrP variants onto the VP1/VP2-pseudotyped VLPs. When the immunized mice were subsequently challenged intraperitoneally with the mouse scrapie strain, the increased mean survival time was observed (Eiden et al. 2021).
Degenerative Diseases of the Nervous System
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
James A. Mastrianni, Elizabeth A. Harris
The prion protein gene (PRNP) blood test is a genetic test for mutations that cause familial prion disease, using DNA analysis from blood or brain. The PRNP gene is sequenced for the presence of pathogenic mutations, and the polymorphic risk factor found at codon 129. It is important to perform, even if no family history is apparent, as mutations are occasionally detected in apparently sporadic cases as a result of incomplete penetrance, nonpaternity, adoption, or because the gene-carrying parent died from another cause at a young age, prior to their manifestation of prion disease.
Pityriasis rubra pilaris induced erythroderma
Published in Biju Vasudevan, Rajesh Verma, Dermatological Emergencies, 2019
There are several case reports of successful use of ustekinumab, the anti-IL-12/IL-23 antibody in refractory cases of PRP [15,31–34]. The mechanism of action in PRP is by suppression of activation of the NF-κB pathway. Marked improvement has been reported in both familial and sporadic cases. Ustekinumab may be considered in preference to TNF-α inhibitors in severe or erythrodermic PRP when acitretin and methotrexate have been ineffective or are contraindicated.
Tackling prion diseases: a review of the patent landscape
Published in Expert Opinion on Therapeutic Patents, 2021
Marco Zattoni, Giuseppe Legname
Human prion diseases are etiologically divided into idiopathic, genetic, and acquired. Among the idiopathic forms, which accounts for the majority of prion diseases cases, there are sporadic Creutzfeldt-Jakob disease (sCJD) and variably protease-sensitive prionopathy. Genetic prion diseases, such as, fatal familial insomnia, genetic CJD and Gerstmann-Sträussler-Scheinker syndrome are characterized by autosomal dominant mutations in human PRNP gene. The acquired forms are very rare and account for ritual cannibalism, as Kuru, contamination through surgical instruments, as in the case of iatrogenic CJD, or consumption of animal products contaminated with the agent responsible for the bovine spongiform encephalopathies (BSE) [7]. The BSE epidemic, often referred to as ‘mad cow disease,’ has attracted the attention of the public health authorities and the scientific community since it was shown to cause a new variant form of Creutzfeldt-Jakob disease (vCJD) in humans [8,9]. Besides BSE, other prion diseases discovered in animals include scrapie in sheep and goats and chronic wasting diseases (CWD) in cervids, for which no transmission to humans has been shown so far, although their potential risk cannot be dismissed [10,11]
Atypical and early symptoms of sporadic Creutzfeldt – Jakob disease: case series and review of the literature
Published in International Journal of Neuroscience, 2021
Grammatiki Katsikaki, Ioannis E. Dagklis, Petros Angelopoulos, Dimitrios Ntantos, Angeliki Prevezianou, Sevasti Bostantjopoulou
Sporadic CJD presenting with symptoms and signs of brainstem involvement is uncommon [132]. Although mild histopathological and immunohistochemical alterations can be found in the brainstem of patients with sCJD, spongiform changes were found only infrequently and abnormal PrPSc depositions are not necessarily associated with clinical symptoms or neuronal loss [133,134]. Isolated dysarthria, dysphagia, vocal cord and soft palate paralysis, lethargy, respiratory failure and central apnea as initial manifestations have been described [135–138]. Moreover, an unique case with hyperekplexia [139] and with vulvodynia [140] have been reported recently. The location of the lesion is not easily identifiable and the distinction of the damage, either as bulbar or as pseudobulbar [141] is based on the findings from the MRI and neurophysiological evaluation.
Platelet rich plasma in oral and maxillofacial surgery from the perspective of composition
Published in Platelets, 2021
Eduardo Anitua, Sofía Fernández-de-Retana, Mohammad H. Alkhraisat
Furthermore, the application of PRP has also been evaluated in the prevention and treatment of medication-related osteonecrosis of the jaw (MRONJ) and osteoradionecrosis). A systematic review concluded that there is not enough evidence to support the application of PRP in the treatment of MRONJ. Importantly, the composition of PRP was not evaluated [35]. Regarding the types of PRP, there is poor evidence supporting the efficacy of L-PRP in the management of these pathological situations. An RCT did not observe any beneficial effect of applying an L-PRP to prevent the occurrence of osteoradionecrosis [36]. Similarly, two non-controlled studies evaluated the efficacy of L-PRP as adjuvant therapy [37,38], reporting success rates ranging from 50% to 83.3%. Finally, another RCT described that the addition of laser therapy and L-PRP to surgical and pharmaceutical treatment of MRONJ resulted in lower bone exposure [39] (Figure 3).