Prion disease and recommended procedures for flexible endoscope reprocessing – review of policies worldwide and proposal for a simplified approach

Publication date: Available online 10 August 2019Source: Journal of Hospital InfectionAuthor(s): Günter Kampf, Michael Jung, Miranda Suchomel, Philippe Saliou, Helen Griffiths, Margreet C. VosSummarySeveral guidelines recommend specific treatments for endoscopes, procedures of quarantine for endoscopes or additional treatments for the endoscope washer disinfector (EWD) in suspected or confirmed cases of CJD or vCJD but vary in many details. We therefore reviewed guidelines on reprocessing flexible endoscopes after use in patients with suspected or confirmed prion disease. In addition, a literature search was done on Medline on prion, CJD, vCJD, chemical inactivation, transmission healthcare, epidemiology healthcare, concentration tissue human and endoscope. So far no case of CJD or vCJD transmitted by flexible endoscope has been reported. In animals it was shown that oral uptake of 0.1 – 5 g of BSE-infected brain homogenate is necessary for transmission. The maximum prion concentration in other tissues (e.g. terminal ileum) is at least 1000-fold lower. Automated cleaning of endoscopes alone results in very low residual protein ≤ 5.6 mg per on duodenoscopes. Recommendations vary between countries, sometimes with additional cleaning, use of alkaline cleaners, no use of cleaners with fixative properties, use of disinfectants without fixative properties or single use disinfectants. Sodium hydroxide (1 M) and sodium hypochlorite (10,000 and 25,000 mg/l) are very effe...
Source: Journal of Hospital Infection - Category: Infectious Diseases Source Type: research

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Diana Paterson is the third person this year to have cataract surgery at the Moncton Hospital, then go on to be diagnosed with Creuztfeld-Jakob disease. An infectious disease doctor says the surgery and the fatal disease aren't connected, and there's no cause for concern.
Source: CBC | Health - Category: Consumer Health News Tags: News/Canada/New Brunswick Source Type: news
Diana Paterson is the third person this year to have cataract surgery at the Moncton Hospital, then go on to be diagnosed with Creutzfeldt-Jakob disease. An infectious disease doctor says the surgery and the fatal disease aren't connected, and there's no cause for concern.
Source: CBC | Health - Category: Consumer Health News Tags: News/Canada/New Brunswick Source Type: news
AbstractCerebrospinal fluid (CSF) non-phosphorylated tau (non-p-tau) is increased in sporadic Creutzfeldt –Jakob disease (CJD), but its accuracy in the differential diagnosis has not been previously established. Here, we first used a retrospective cohort of non-CJD (n = 135) and CJD (n = 137) cases to determine the optimal cutoff point for the discrimination of CJD cases. Next, we prospectively quantified non-p-tau and 14-3-3 protein in a cohort of 1427 cases received for CSF testing at the German National Reference Center for transmissible spongiform encephalopathies. Among them, 36 were ...
Source: Journal of Neurology - Category: Neurology Source Type: research
DISCUSSION: Biomarkers of synapse and neurodegeneration differentiate HC subjects from neurodegenerative dementias and between AD, FTD, and CJD. NF-L and 14-3-3 performed similar to total tau when AT(N) system was applied. PMID: 31668967 [PubMed - as supplied by publisher]
Source: The Journal of Alzheimers Association - Category: Psychiatry Tags: Alzheimers Dement Source Type: research
This study retrospectively reviewed 26 cases of sCJD diagnosed in a single center in recent years. All 26 sCJD patients received brain magnetic resonance imaging scan, cerebrospinal fluid 14-3-3 protein detection, electroencephalogram, and PRNP gene screening. The codon 129 polymorphism were all homozygous MM in 26 sCJD patients. The main onset symptoms of sCJD patients were rapidly progressive dementia, visual impairment, and cerebellar ataxia. At the time of diagnosis, the incidence of myoclonus and akinetic mutism were relatively low (
Source: Alzheimer Disease and Associated Disorders - Category: Psychiatry Tags: Alzheimer Dis Assoc Disord Source Type: research
Publication date: Available online 25 October 2019Source: NeuroImage: ClinicalAuthor(s): Liane S. Canas, Carole H. Sudre, Enrico De Vita, Akin Nihat, Tze How Mok, Catherine F. Slattery, Ross W. Paterson, Alexander J.M. Foulkes, Harpreet Hyare, M. Jorge Cardoso, John Thornton, Jonathan M. Schott, Frederik Barkhof, John Collinge, Sébastien Ourselin, Simon Mead, Marc ModatAbstractPrion diseases are a group of rare neurodegenerative conditions characterised by a high rate of progression and highly heterogeneous phenotypes. Whilst the most common form of prion disease occurs sporadically (sporadic Creutzfeldt-Jakob disea...
Source: NeuroImage: Clinical - Category: Radiology Source Type: research
DiscussionBiomarkers of synapse and neurodegeneration differentiate HC subjects from neurodegenerative dementias and between AD, FTD, and CJD. NF-L and 14-3-3 performed similar to total tau when AT(N) system was applied.
Source: Alzheimer's and Dementia: The Journal of the Alzheimer's Association - Category: Geriatrics Source Type: research
In this study we describe, a large Indian family with familial Creutzfeldt Jakob Disease (fCJD). One affected member presented with a presenile dementia, a protracted clinical course and characateristic MRI features. Genetic analysis revealed a D178N mutation in the 2 affected individuals and 7 unaffected members. The neuropathological examination of the brain of one of the affected member was conspicuous by spongiform degeneration, neuronal loss and gliosis. This is a detailed report of a genetically and neuropathologically proven fCJD from India.
Source: Annals of Indian Academy of Neurology - Category: Neurology Authors: Source Type: research
In this study, we attempted to clinically predict the concurrence of MM-type sCJD with another PrPSc type in the same individual. We retrospectively identified seven MM-type sCJD cases with both fine vacuole-type spongiform (FV) and large confluent vacuole-type spongiform change (LCV) among 49 sCJD cases.
Source: Journal of the Neurological Sciences - Category: Neurology Authors: Source Type: research
Abstract PrPC is a host-encoded prion protein which gets post translationally modified into a transmissible, β-sheet rich disease associated protein called PrPSc, responsible for the Prion disease including mad cow disease in cattles and CJD in humans. The PrP 106-126 region in PrPSc peptide initiates the conformational change in that protein leading fibrillation. Any agent that can destabilize or disintegrate such proteins can be served as a potential drug candidate for Prion diseases. In the present study, an enzyme Lumbrokinase (LK) was isolated from earthworm and its activity was exploited towards PrP 106...
Source: CNS and Neurological Disorders Drug Targets - Category: Drugs & Pharmacology Authors: Tags: CNS Neurol Disord Drug Targets Source Type: research
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