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MedWorm: Mad Cow Disease



MedWorm.com provides a medical RSS filtering service. Over 7000 RSS medical sources are combined and output via different filters. This feed contains the latest news and research in the Mad Cow Disease category.



Last Build Date: Tue, 29 Mar 2016 16:31:35 +0100

 



Dura mater is a potential source of Aβ seeds

Thu, 24 Mar 2016 23:00:00 +0100

Abstract Deposition of amyloid-β (Aβ) in the brain parenchyma and vessels is one of the hallmarks of Alzheimer disease (AD). Recent observations of Aβ deposition in iatrogenic Creutzfeldt-Jakob disease (iCJD) after dural grafting or treatment with pituitary extracts raised concerns whether Aβ is capable of transmitting disease as seen in prion diseases by the disease-associated prion protein. To address this issue, we re-sampled and re-evaluated archival material, including the grafted dura mater of two cases with iCJD (28 and 33-years-old) without mutations in the AβPP, PSEN1 and PSEN2 genes, and carrying ε3/ε3 alleles of the APOE gene. In addition, we evaluated 84 dura mater samples obtained at autopsy (mean age 84.9 ± 0.3) in the community-based VITA study for the pre...

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France confirms case of mad cow disease

Thu, 24 Mar 2016 11:42:01 +0100

PARIS (Reuters) - France's agriculture ministry confirmed on Thursday that a case of bovine spongiform encephalopathy (BSE), or mad cow disease, had been discovered in the northeastern region of Ardennes. (Source: Reuters: Health)



CSF biomarkers in neurodegenerative and vascular dementias

Tue, 22 Mar 2016 23:00:00 +0100

Publication date: Available online 22 March 2016 Source:Progress in Neurobiology Author(s): Franc Llorens, Matthias Schmitz, Isidro Ferrer, Inga Zerr Neurodegenerative diseases with abnormal protein aggregates such as Alzheimer’s disease, tauopathies, synucleinopathies, and prionopathies, together with vascular encephalopathies, are cause of cognitive impairment and dementia. Identification of reliable biomarkers in biological fluids, particularly in the cerebrospinal fluid (CSF), is of extreme importance in optimizing the precise early clinical diagnosis of distinct entities and predicting the outcome in particular settings. In addition, the study of CSF biomarkers is useful to identify and monitor the underlying pathological processes developing in the central nervous system of a...



Fayetteville hospital fights Creutzfeldt-Jakob disease

Thu, 17 Mar 2016 08:47:12 +0100

An Arkansas hospital shut down its operating rooms and sterilized surgical equipment after a possible diagnosis of a rare brain disease, state health officials said. The disease, Creutzfeldt-Jakob, was detected in a patient at Washington Regional Medi... (Source: WDSU.com - Health)



A toxic byproduct of hemoglobin could provide treatments for Creutzfeldt-Jakob disease

Tue, 08 Mar 2016 13:00:00 +0100

Scientists at Case Western Reserve University School of Medicine have identified a novel mechanism that could be used to protect the brain from damage due to stroke and a variety of... (Source: Parkinson's Disease News From Medical News Today)



Integrating patient reported measures as predictive parameters into decisionmaking about palliative chemotherapy: a pilot study

Tue, 01 Mar 2016 00:00:00 +0100

Systemic treatment has proven to improve physical symptoms in patients with advanced cancer. Relationship between quality of life (QoL) or symptom burden (SYB) and treatment efficacy (tumour response and survi... (Source: BMC Palliative Care)

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CJD: Understanding Creutzfeldt-Jakob disease

Thu, 25 Feb 2016 17:36:15 +0100

No abstract available (Source: Nursing)



Understanding Creutzfeldt-Jakob disease

Thu, 25 Feb 2016 17:36:15 +0100

No abstract available (Source: Nursing)



Trial Simulations and Outcome Measures in Creutzfeldt-Jakob Disease

Mon, 22 Feb 2016 00:00:00 +0100

This interval-cohort study aims to establish a more powerful and meaningful clinical trial method in sporadic Creutzfeldt-Jakob disease. (Source: JAMA Neurology)



Systematic reviews in paediatric multiple sclerosis and Creutzfeldt-Jakob disease exemplify shortcomings in methods used to evaluate therapies in rare conditions

Sat, 20 Feb 2016 00:00:00 +0100

Randomized controlled trials (RCTs) are the gold standard design of clinical research to assess interventions. However, RCTs cannot always be applied for practical or ethical reasons. To investigate the curren... (Source: Orphanet Journal of Rare Diseases)



Neurologists as primary palliative care providers: Communication and practice approaches

Mon, 15 Feb 2016 00:00:00 +0100

Purpose of review: To present current knowledge and recommendations regarding communication tasks and practice approaches for neurologists as they practice primary palliative care, including discussing serious news, managing symptoms, aligning treatment with patient preferences, introducing hospice/terminal care, and using the multiprofessional approach. Recent findings: Neurologists receive little formal palliative care training yet often need to discuss prognosis in serious illness, manage intractable symptoms in chronic progressive disease, and alleviate suffering for patients and their families. Because patients with neurologic disorders often have major cognitive impairment, physical impairment, or both, with an uncertain prognosis, their palliative care needs are particularly challe...

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ID 322 – Familial Creutzfeldt-Jakob disease. All night v-polysomnography study

Thu, 11 Feb 2016 06:19:56 +0100

We describe the sleep structure assessed by night v-polysomnography in a patient with Glu200Lys mutation. (Source: Clinical Neurophysiology)



Amyloid-β pathology and cerebral amyloid angiopathy are frequent in iatrogenic Creutzfeldt-Jakob disease after dural grafting.

Wed, 27 Jan 2016 17:57:03 +0100

CONCLUSIONS: We conclude that congophilic amyloid angiopathy and brain parenchymal Aβ plaques are frequent in iCJD after dural grafting. The presence of Aβ pathology in young individuals is highly unusual and suggests a causal relationship to the dural grafts. Further studies will be needed to elucidate whether such pathology resulted from the seeding of Aβ aggregates from the grafts to host tissues. PMID: 26812492 [PubMed - as supplied by publisher] (Source: Swiss Medical Weekly)



Autopsies suggest surgical grafts passed on Alzheimer’s disease

Wed, 27 Jan 2016 17:28:37 +0100

There is growing evidence that Alzheimer's disease was spread via surgical grafts as well as growth hormone donations several decades ago (Source: New Scientist - Health)



New clues that Alzheimer’s may have been spread during surgery

Wed, 27 Jan 2016 13:30:00 +0100

ConclusionThis latest research adds some evidence to the possibility that amyloid beta proteins could have been passed on during certain types of treatment, which introduced substances derived from donor brains or pituitary glands into the body. However, these types of treatment are no longer used. The theory is far from certain, and other possible causes need to be investigated. Even if the theory was proven, we don't know that having these proteins introduced into the brain in this way would cause Alzheimer's disease. All the evidence showing amyloid protein in the brain after medical treatment has come from studies of the brains of people known to have been infected with prions causing CJD. None of these people actually showed outward signs of Alzheimer's. There is absolutely no need to...



Mitochondrial DNA differentiates Alzheimer from Creutzfeldt-Jakob disease.

Wed, 27 Jan 2016 05:57:03 +0100

DISCUSSION: Low-CSF mtDNA is not a consequence of brain damage and allows the differential diagnosis of AD from sCJD and other dementias. These results support the hypothesis that mtDNA in CSF is a pathophysiological biomarker of AD. PMID: 26806388 [PubMed - as supplied by publisher] (Source: The Journal of Alzheimers Association)

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Mitochondrial DNA differentiates Alzheimer from Creutzfeldt-Jakob disease

Sat, 23 Jan 2016 00:00:00 +0100

Discussion Low-CSF mtDNA is not a consequence of brain damage and allows the differential diagnosis of AD from sCJD and other dementias. These results support the hypothesis that mtDNA in CSF is a pathophysiological biomarker of AD. (Source: Alzheimer's and Dementia: The Journal of the Alzheimer's Association)



L-Arginine ethylester enhances in vitro amplification of PrP(Sc) in macaques with atypical L-type bovine spongiform encephalopathy and enables presymptomatic detection of PrP(Sc) in the bodily fluids.

Wed, 20 Jan 2016 00:00:00 +0100

Authors: Murayama Y, Ono F, Shimozaki N, Shibata H Abstract Protease-resistant, misfolded isoforms (PrP(Sc)) of a normal cellular prion protein (PrP(C)) in the bodily fluids, including blood, urine, and saliva, are expected to be useful diagnostic markers of prion diseases, and nonhuman primate models are suited for performing valid diagnostic tests for human Creutzfeldt-Jakob disease (CJD). We developed an effective amplification method for PrP(Sc) derived from macaques infected with the atypical L-type bovine spongiform encephalopathy (L-BSE) prion by using mouse brain homogenate as a substrate in the presence of polyanions and L-arginine ethylester. This method was highly sensitive and detected PrP(Sc) in infected brain homogenate diluted up to 10(10) by sequential amplification...



Neotropical primate nursery in a squirrel monkey breeding unit in Brazil

Tue, 19 Jan 2016 00:00:00 +0100

Saimiri (squirrel monkey) is a neotropical primate of the Simian genus that has been bred in captivity for the development of research into human and animal health. They have been widely used in studies in ophthalmology, toxicology, pharmacology, psychiatry, neuroscience, vaccines and drug tests (such as malaria and measles agents), as well as effects on interactive behavior and cognition of Creutzfeldt-Jakob disease in man. The main concern of non-human primate (NHP) research centers is focused on the establishment of self-sustaining breeding colonies providing good quality research animals. Maternal rejection, dystocia and pneumonia are the main causes of newborn deaths in these species. Therefore, in order to ensure the survival of these valuable animals, the Laboratory Animals Breeding...



Visual art therapy in sporadic Creutzfeldt-Jakob disease: a case study.

Mon, 18 Jan 2016 00:00:00 +0100

Authors: Shrestha R, Trauger-Querry B, Loughrin A, Appleby BS Abstract This paper describes the diagnostic and treatment utility of visual art therapy in a case of sporadic Creutzfeldt-Jakob disease. Visual art therapy was compared longitudinally with clinical and neuroimaging data over five-month period in an autopsy-confirmed case of sporadic Creutzfeldt-Jakob disease of MM2-cortical subtype. Art therapy sessions and content were useful in ascertaining neuropsychiatric symptoms during the course of her illness. Art therapy offered a unique emotional and cognitive outlet as illness progressed. Patients and families affected by sporadic Creutzfeldt-Jakob disease may benefit from art therapy despite the rapidly progressive nature of the illness. Art therapy can also be useful for as...



Comparative analysis of cerebrospinal fluid biomarkers in the differential diagnosis of neurodegenerative dementia.

Sat, 16 Jan 2016 18:52:02 +0100

CONCLUSION: Combining the biomarker panel allows differentiating between various types of neurodegenerative dementias and contributes to a better understanding of their pathophysiological processes. PMID: 26718584 [PubMed - as supplied by publisher] (Source: The Journal of Alzheimers Association)

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Strain-Specific Altered Regulatory Response of Rab7a and Tau in Creutzfeldt-Jakob Disease and Alzheimer’s Disease

Thu, 14 Jan 2016 00:00:00 +0100

In this study, we performed a targeted search for endocytic pathway proteins in the different regions of the brain. We found late endosome marker Rab7a which was significantly upregulated in the frontal cortex region in the rapid progressive CJD form (MM1) and rapid progressive AD (rpAD) forms. However, Rab9 expression was significantly downregulated only in CJD-MM1 brain frontal cortex region. In the cerebellum, Rab7a expression showed significant upregulation in both subtype MM1 and VV2 CJD forms, in contrast to Rab9 which showed significant downregulation in both subtype MM1 and VV2 CJD forms at terminal stage of the disease. To check regulatory response at pre-symptomatic stage of the disease, we checked the regulatory interactive response of Rab7a, Rab9, and known biomarkers PrPC and ...



Creutzfeldt–Jakob disease and blood transfusion: updated results of the UK Transfusion Medicine Epidemiology Review Study

Mon, 28 Dec 2015 00:00:00 +0100

ConclusionThe results of the ongoing TMER study show no new cases of transfusion‐associated vCJD since 2007 and no evidence of transfusion transmission of sCJD. (Source: Vox Sanguinis)



Behind the Headlines' Top Five of Top Fives 2015

Thu, 24 Dec 2015 15:00:00 +0100

In this study, researchers wanted to see why this is and if there could be any human applications.Researchers collected white blood cells from African and Asian elephants. They found that elephants have at least 20 copies of a gene called TP53. TP53 is known to encourage cell "suicide" when DNA is damaged, stopping any potential cancer in its tracks. In contrast, humans are thought to have only a single copy of the TP53 gene.Of course the big question – the elephant in the room, if you will – is how we can boost TP53 activity in humans to stimulate a similar protective effect. The simple answer is: we don't know. Yet. Researchers 'a step closer' to universal flu vaccineIt's just like buses. You wait years for a potential universal flu vaccine, then two come along at once. In ...



Behind the Headlines Top Five of Top Fives 2015

Thu, 24 Dec 2015 15:00:00 +0100

In this study, researchers wanted to see why this is and if there could be any human applications. Researchers collected white blood cells from African and Asian elephants. They found elephants have at least 20 copies of a gene called TP53. TP53 is known to encourage cell "suicide" when DNA is damaged, stopping any potential cancer in its tracks. In contrast, humans are thought to have only a single copy of the TP53 gene. Of course the big question – the elephant in the room, if you will – is how we can boost TP53 activity in humans to stimulate a similar protective effect. The simple answer is: we don't know. Yet. Researchers 'a step closer' to universal flu vaccine It just like buses. You wait years for a potential universal flu vaccine then two come along at the same time...



Comparative analysis of cerebrospinal fluid biomarkers in the differential diagnosis of neurodegenerative dementia

Tue, 22 Dec 2015 00:00:00 +0100

Conclusion Combining the biomarker panel allows differentiating between various types of neurodegenerative dementias and contributes to a better understanding of their pathophysiological processes. (Source: Alzheimer's and Dementia: The Journal of the Alzheimer's Association)

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[Pulvinar].

Sat, 12 Dec 2015 08:37:21 +0100

Authors: Szirmai I Abstract The pulvinar is the largest nucleus of the thalamus. Its lateral and inferior areas have rich connections with the visual- and dorsolateral parietal cortices. Several cells in the medial and upper area connect the anterior cingulum and the premotor and prefrontal association areas. This neuronal network was considered to organize the saccades and visual attention. Other cells in the medial nucleus have axonal connections with paralimbic-, insular and higher order association-cortices. The medial structure integrates complex sensory information with limbic reactivity settings, transmitting these to the temporal and parieto-occipital centres. The pulvinar is supplied by the posterior chorioideal artery. Visual salience is considered to be an important func...



Apolipoprotein E gene polymorphism may increase susceptibility to sporadic Creutzfeldt-Jakob disease in a Korean population

Tue, 08 Dec 2015 00:00:00 +0100

Creutzfeldt-Jakob disease (CJD) and Alzheimer's disease are neurodegenerative disorders that share a pathologic mechanism involving amyloidosis in the brain and clinical signs of dementia. The apolipoprotein E (APOE) 4 allele and its homozygosity are well-established risk factors for Alzheimer's disease. However, their association with CJD has been controversial. To investigate whether the APOE gene polymorphism is a risk factor for developing sporadic CJD, we analyzed genotype and allele frequencies of the gene in 230 patients with sporadic CJD and 207 healthy individuals. (Source: Neurobiology of Aging)



WITHDRAWN: Apolipoprotein E gene polymorphism may increase susceptibility to sporadic Creutzfeldt-Jakob disease in a Korean population

Tue, 08 Dec 2015 00:00:00 +0100

Available online 8 December 2015 (Source: Neurobiology of Aging)



Hyperekplexia as the presenting symptom of Creutzfeldt-Jakob disease

Mon, 07 Dec 2015 00:00:00 +0100

We describe a patient with hyperekplexia as the initial presenting symptom in sporadic Creutzfeldt-Jakob disease (CJD). We also demonstrate the utility of the sensitive and highly specific new prion protein amplification assay, real-time quaking-induced conversion (RT-QUIC), which amplifies and detects misfolded β-sheet–rich conformers of the prion protein (PrPsc), in expediting a confident clinical diagnosis of CJD in an atypical case. (Source: Nature Clinical Practice)



Sporadic Creutzfeldt-Jakob disease in a native Puerto Rican patient.

Wed, 02 Dec 2015 13:25:29 +0100

We report the case of a 56-year old patient native to Puerto Rico suspected of sporadic Creutzfeldt-Jakob disease (sCD). The symptoms at onset were notorious for bilateral cortical blindness followed by rapidly progressive cognitive decline, visual deficit, increased levels of CSF 14-3-3 and tau along with positive brain MRI and EEG, are highly indicative of CJD. The definite diagnosis was confirmed by the National Prion Disease Pathology Surveillance Center (NPDPSC), in Cleveland, Ohio, USA. Lack of genetic mutations in the prion protein (PrP) gene, widespread histopathological changes and the accumulation of scrapie PrP (PrPSc) in the brain confirmed the diagnosis of sCJD. The patient, admitted to our institution in 2011, represents the first detailed report of sCJD in a native Puerto Ri...

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Overview and evaluation of 15 years of Creutzfeldt-Jakob disease surveillance in Belgium, 1998-2012

Wed, 02 Dec 2015 00:00:00 +0100

Conclusions: Although 40 % of the suspect CJD cases were not referred for autopsy, our data suggest that the Belgian CJD surveillance system meets one of its main objectives: it can detect changes in CJD incidence. However, we do not have sufficient evidence to conclude that the system meets its second objective of detecting vCJD cases arising in Belgium. Although not well known, the system was considered acceptable by its stakeholders. (Source: BMC Neurology)



Prion Diseases

Tue, 01 Dec 2015 06:00:00 +0100

This article presents an update on the clinical aspects of human prion disease, including the wide spectrum of their presentations. Recent Findings:: Prion diseases, a group of disorders caused by abnormally shaped proteins called prions, occur in sporadic (Jakob-Creutzfeldt disease), genetic (genetic Jakob-Creutzfeldt disease, Gerstmann-Stra¨ussler-Scheinker syndrome, and fatal familial insomnia), and acquired (kuru, variant Jakob-Creutzfeldt disease, and iatrogenic Jakob-Creutzfeldt disease) forms. This article presents updated information on the clinical features and diagnostic methods for human prion diseases. New antemortem potential diagnostic tests based on amplifying prions in order to detect them are showing very high specificity. Understanding of the diversity of possible presen...



Rapid cognitive decline: not always Creutzfeldt-Jakob disease.

Mon, 30 Nov 2015 21:38:02 +0100

Authors: Randall A, Ellis R, Hywel B, Davies RR, Alusi SH, Larner AJ Abstract A patient with rapidly progressive cognitive decline over an approximately four month period was suspected to have sporadic Creutzfeldt-Jakob disease. Features thought to support this diagnosis included psychiatric symptoms (anxiety and depression), visual hallucinations and a visual field defect. However, the finding of papilloedema broadened the differential diagnosis. Although standard brain imaging and electroencephalography had shown only non-specific abnormalities, subsequent cerebral angiography disclosed an intracranial dural arteriovenous fistula. Following embolisation, the patient made a good functional recovery. Intracranial dural arteriovenous fistula merits consideration in any patient with ...



Donating blood for research: a potential method for enhancing customer satisfaction of permanently deferred blood donors.

Fri, 27 Nov 2015 00:00:00 +0100

This study examined attitudes towards donating blood for research use in a sample of permanently deferred Australian donors. MATERIALS AND METHODS: Donors permanently deferred because of a risk of variant Creutzfeldt-Jakob disease (n=449) completed a postal survey that examined attitudes towards research donation. RESULTS: The majority of participants were interested in donating blood for research (96%), and joining a registry of research donors (93%). Participants preferred to donate for transfusion or clinical research, and were willing to travel large distances. Results indicated that positive attitudes towards the Blood Service would be extended if the opportunity to donate blood was provided. These findings indicate a desire for continued engagement with the Blood Service desp...



Blood transmission studies of prion infectivity in the squirrel monkey (Saimiri sciureus): the Baxter study.

Mon, 23 Nov 2015 00:00:00 +0100

CONCLUSION: Blood transmits GSS but not sCJD or vCJD infectivity to IC- or IV-inoculated squirrel monkeys within a 7-year observation period. PMID: 26594017 [PubMed - as supplied by publisher] (Source: Transfusion)

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Intracranial Procedures and Expected Frequency of Creutzfeldt-Jakob Disease

Thu, 19 Nov 2015 23:50:58 +0100

Conclusions: The considerable proportion of US residents living with a history of ICP is important information for retrospective assessments of CJD or any other suspected long-term outcome of ICPs.Neuroepidemiology 2016;46:1-8 (Source: Neuroepidemiology)



An experiential learning model applied to nurses working with patients with Creutzfeldt-Jakob disease.

Wed, 18 Nov 2015 22:37:41 +0100

Authors: D'Amour R, Guimond P Abstract Creutzfeldt-Jacob disease (C/D) is a rare neurological disease, transmissible, incurable and always fatal affecting humans, as well as animals. In the 1980s, the "mad cow disease" (MCD) epidemic in the United Kingdom popularized prion diseases worldwide. However, this contributed to the proliferation of disinformation, causing confusion between C/D and MCD in the public, as well as in some health care providers. The purpose of this article is to describe the process utilized to develop, implement, and evaluate a workshop on CJD for nurses and other health care providers. Kolb's experiential teaching/learning model was used as a framework for this workshop. A workbook was developed to complement the participants' learning. Fifteen health care p...



Evolution of DWMRI Signal Abnormality in Creutzfeldt-Jakob Disease

Mon, 16 Nov 2015 00:00:00 +0100

This study assesses gray matter involvement on diffusion-weighted imaging in patients with sporadic Creutzfeldt-Jakob disease and investigates the potential of these images as a biomarker of disease progression. (Source: JAMA Neurology)



Dysfunction of glutamate receptors in microglia may cause neurodegeneration.

Mon, 16 Nov 2015 00:00:00 +0100

Authors: Noda M Abstract Dysregulation of glutamate signalling is important in Alzheimer's disease and other pathologies. There has been a focus on changes in neuronal glutamate signalling, but microglia also express glutamate receptors (GluRs), which are known to modulate their responses to neuropathology. Microglia express both metabotropic and ionotropic GluRs. Among ionotropic GluRs, microglial AMPA (-amino-hydroxy-5-methyl-isoxazole-4-propionate)-type of GluRs (AMPA-Rs) are Ca2+ impermeable due to the expression of subunit GluA2. Upon activation of microglia, expression level of surface GluA2 subunits significantly increase, while expression of GluA1, A3 and A4 subunits on membrane surface significantly decrease. Owing to the GluA2 subunits-dominant composition, AMPA-Rs in ...



Pathologically confirmed autoimmune encephalitis in suspected Creutzfeldt-Jakob disease

Thu, 12 Nov 2015 00:00:00 +0100

Conclusions: It is important to consider immune-mediated disorders in the differential diagnosis of rapidly progressive neurologic deficits. (Source: Neurology Neuroimmunology and Neuroinflammation)

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Teaching NeuroImages: Recurrent SSPE presenting as Anton syndrome with cortical ribboning

Mon, 09 Nov 2015 00:00:00 +0100

A 21-year-old man, an immigrant from India who had not been vaccinated against measles in childhood, presented with new visual loss, on the background of a 4-year history of cognitive impairment due to subacute sclerosing panencephalitis (SSPE). SSPE was previously managed with 3 times weekly intrathecal α-interferon, which was stopped 18 months previously given stable disease. Examination confirmed baseline cognitive impairment. Vision was reduced to light perception, although the patient denied visual loss. Brain MRI showed occipital cortex diffusion restriction (figure). CSF measles titers were elevated. The patient was diagnosed with Anton syndrome due to SSPE. Off-label intrathecal α-interferon was restarted, recognizing prior effect in this patient and reports of improvem...



Real-Time Quaking-Induced Conversion Analysis for the Diagnosis of Sporadic Creutzfeldt-Jakob Disease in Korea.

Sat, 07 Nov 2015 18:28:12 +0100

CONCLUSIONS: The sensitivity of the RT-QuIC in this study was similar to that in some previous reports, and the specificity of RT-QuIC was higher than that of 14-3-3 in CSF, suggesting that RT-QuIC analysis can complement the weakness of the specificity of 14-3-3 for the diagnosis of sCJD. These results indicate that RT-QuIC might be very useful for the rapid and specific diagnosis of sCJD and provide a practical novel method for the ante-mortem diagnosis of human prion diseases. PMID: 26541494 [PubMed - as supplied by publisher] (Source: Journal of Clinical Neurology)



Neuron-Specific Enolase as a Biomarker: Biochemical and Clinical Aspects.

Fri, 06 Nov 2015 21:46:02 +0100

Authors: Isgrò MA, Bottoni P, Scatena R Abstract Neuron-specific enolase (NSE) is known to be a cell specific isoenzyme of the glycolytic enzyme enolase. In vertebrate organisms three isozymes of enolase, expressed by different genes, are present: enolase α is ubiquitous; enolase β is muscle-specific and enolase γ is neuron-specific. The expression of NSE, which occurs as γγ- and αγ-dimer, is a late event in neural differentiation, thus making it a useful index of neural maturation.NSE is a highly specific marker for neurons and peripheral neuroendocrine cells. As a result of the findings of NSE in specific tissues under normal conditions, increased body fluids levels of NSE may occur with malignant proliferation and thus can be of value in diagnosis, staging and treatment ...



Inside The NASA Mission To Answer 'What Is Life?'

Fri, 06 Nov 2015 19:12:19 +0100

“Why would NASA want to study a lake in Canada?” Three different border guards asked me variations on that question, and while they ultimately let me pass, it was obvious they didn’t understand. Why is NASA interested in a lake in Canada? And what business is it of mine? As exotic environments go, Pavilion Lake in British Columbia is rather ordinary. Certainly it’s remote – the closest major city is Vancouver, a long drive away over the mountains. The closest towns are light dustings of houses over the dry slopes, and the road winds for dozens of kilometers of empty desert country between them. The lake itself lies along a paved highway, and from the road it doesn’t look different to any other modestly sized mountain lake in western North America. But be...



No evidence of asymptomatic variant CJD infection in immunodeficiency patients treated with UK‐sourced immunoglobulin

Tue, 03 Nov 2015 00:00:00 +0100

Surveillance of 75 immunodeficiency patients exposed to UK‐sourced immunoglobulin, including batches derived from donors who went on to develop vCJD, has not detected any clinical cases of vCJD, or of asymptomatic infection in 15 patients with available tissue samples of sufficient quality for testing. (Source: Vox Sanguinis)



Clinicopathological Correlations and Concomitant Pathologies in Rapidly Progressive Dementia: A Brain Bank Series

Mon, 02 Nov 2015 23:42:52 +0100

Conclusions: Prion diseases were accurately identified in our series. In contrast, non-prion RPD diagnosis was poor while the patients were still alive, supporting the need for better diagnostic tools and confirmatory neuropathological studies. The presence of concomitant AD pathology in RPD should be taken into account in the interpretation of amyloid biomarkers.Neurodegener Dis (Source: Neurodegenerative Diseases)



Clinicopathological Correlations and Concomitant Pathologies in Rapidly Progressive Dementia: A Brain Bank Series.

Sat, 31 Oct 2015 00:00:00 +0100

CONCLUSIONS: Prion diseases were accurately identified in our series. In contrast, non-prion RPD diagnosis was poor while the patients were still alive, supporting the need for better diagnostic tools and confirmatory neuropathological studies. The presence of concomitant AD pathology in RPD should be taken into account in the interpretation of amyloid biomarkers. PMID: 26523804 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)



Genetic Creutzfeldt-Jakob disease mimicking chronic inflammatory demyelinating polyneuropathy

Thu, 29 Oct 2015 00:00:00 +0100

Creutzfeldt-Jakob disease (CJD) is a relentlessly progressive neurodegenerative disorder, belonging to the transmissible spongiform encephalopathies. It is characterized by the deposition of the abnormal isoform of the prion protein (PrPsc), causing spongiform neurodegeneration. The disease is classically known to mainly affect the gray matter of the CNS. However, involvement of the peripheral nervous system (PNS) has been described.1–3 Herein, we describe a patient with genetic CJD, in whom the disease presentation was a demyelinating neuropathy reminiscent of chronic inflammatory demyelinating polyneuropathy (CIDP). (Source: Neurology Neuroimmunology and Neuroinflammation)



High rate of hypoglycemia in 6770 type 2 diabetes patients with comorbid dementia: A multicenter cohort study on 215,932 patients from the German/Austrian diabetes registry

Thu, 29 Oct 2015 00:00:00 +0100

One major public health problem in older people is the occurrence of neurocognitive disorders such as dementia. The prevalence of dementia has increased over the last years, especially in people 85 years and older [1]. It is estimated that globally 35.6 million people live with dementia [2]. The most common type of dementia is Alzheimer's disease [3]. It accounts for 60% to 80% of cases [3]. Other forms of dementia are: (i) vascular dementia, (ii) mixed forms of dementia, (iii) Lewy body dementia, (iv) frontotemporal dementia, and (v) dementia caused by other degenerative conditions (e.g., Creutzfeldt-Jakob disease, Parkinson's disease, normal pressure hydrocephalus) [3]. (Source: Diabetes Research and Clinical Practice)

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More Than a Body of Knowledge

Wed, 28 Oct 2015 18:00:57 +0100

Earlier this month, researchers at Emory University issued a warning that the U.S. could be at risk of yet another measles outbreak. Although the disease is highly contagious and potentially fatal, measles is also highly preventable - if, an individual is vaccinated. Unfortunately, not everyone understands this or the basic science behind immunizations. Why? Because there is a significant lack of scientific literacy throughout the country. For far too many people, the word "science" itself can be distancing. It conjures up stereotypical caricatures of turgid textbooks, disheveled and incoherent adults in lab coats writing on blackboards in musty labs, and other equally stodgy images. In reality though, science and its impact on us is inescapable. Science is everywhere: look around you - w...



Are Prions behind All Neurodegenerative Diseases?

Wed, 28 Oct 2015 15:00:00 +0100

Evidence mounts that chain reactions involving toxic proteins link Creutzfeldt–Jakob disease, Alzheimer’s, Parkinson’s and more -- Read more on ScientificAmerican.com (Source: Scientific American - Official RSS Feed)



Iatrogenic CJD due to pituitary-derived growth hormone with genetically determined incubation times of up to 40 years

Mon, 26 Oct 2015 00:00:00 +0100

We describe the clinical, imaging, molecular and autopsy features in 22 of 24 patients who have developed iatrogenic Creutzfeldt-Jakob disease in the UK since 2003. Mean age at onset of symptoms was 42.7 years. Gait ataxia and lower limb dysaesthesiae were the most frequent presenting symptoms. All had cerebellar signs, and the majority had myoclonus and lower limb pyramidal signs, with relatively preserved cognitive function, when first seen. There was a progressive decline in neurological and cognitive function leading to death after 5–32 (mean 14) months. Despite incubation periods approaching 40 years, the clinical duration in methionine homozygote patients appeared to be shorter than that seen in heterozygote patients. MRI showed restricted diffusion in the basal ganglia, thalam...



Sporadic creutzfeldt-jakob disease presenting with a 'jerky alien limb'

Wed, 14 Oct 2015 00:00:00 +0100

A 64 yr old right-handed mechanic presented with four weeks history of clumsy right hand with ongoing problems at work. He described stiffness and problems with co-ordination. At the time of presentation he was noted to be walking with his right arm behind him and not being aware of this. His initial examination showed Mini Mental Score of 26/30, with ongoing involuntary movements of his right arm (Video). Video shows spontaneous elevation of right arm with occasional myoclonic jerks. Video also demonstrates patient's difficulty in following simple motor tasks and his comments that his arm "has a mind of its own". His MRI head (Figure 1) showed typical cortical ribboning pattern described in sporadic CJD and CSF Protein for 14-3-3, s100b and RT-QUIC findings were consistent with the d...



Variant Creutzfeldt-Jakob disease in older patients

Wed, 14 Oct 2015 00:00:00 +0100

Variant Creutzfeldt-Jakob disease (vCJD) is a zoonotic disease caused by cross-species transmission of bovine spongiform encephalopathy with a median age at onset of 27 years. In 1996, when the first cases were described, a clinicopathological phenotype distinct from sporadic CJD was defined and diagnostic criteria with a high sensitivity and specificity for vCJD have been validated.1 All definite and probable cases of vCJD (201/218) have been methionine homozygous (MM) at codon 129 of the prion protein gene (PRNP). If cases with the other PRNP-129 genotypes (MV, VV) have longer incubation periods, and are exposed at the same age, they will be older by the time they develop symptomatic disease. In addition, patients over 50 are more likely undergo surgical procedures and receive blood...

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Foodborne-Transmitted Prions From the Brain of Cows With Bovine Spongiform Encephalopathy Ascend in Afferent Neurons to the Simian Central Nervous System and Spread to Tonsils and Spleen at a Late Stage of the Incubation Period

Mon, 12 Oct 2015 00:00:00 +0100

Conclusions. Unlike in rodents and ruminants, foodborne BSE-associated prions entered the simian CNS via afferent neurons. From sites of initial CNS invasion, prions spread centrifugally to tonsils and spleen at an advanced stage of the incubation period, thus explaining why tonsil specimens were not reliable for detection of simian disease carriers before onset of clinical signs. (Source: The Journal of Infectious Diseases)



Predicting decline and survival in severe acute brain injury: the fourth trajectory - Creutzfeldt CJ, Longstreth WT, Holloway RG.

Sat, 10 Oct 2015 00:33:54 +0100

Illness trajectories depicting how function declines to death with certain diseases, such as cancer, can help with palliative care. Creutzfeldt and colleagues propose a fourth trajectory is needed for patients with severe acute brain injury to improve d... (Source: SafetyLit: All (Unduplicated))



Serum uric acid and lipid profiles in sporadic Creutzfeldt-Jakob disease.

Wed, 07 Oct 2015 00:00:00 +0100

CONCLUSIONS: The low serum uric acid and LDL/HDL ratio levels in sporadic CJD indicate that dysfunction in the lipid homeostasis and oxidative stress are associated with sporadic prion disease. PMID: 26454226 [PubMed - as supplied by publisher] (Source: Clinical Biochemistry)



Welsh government reports 'mad cow' case, says no risk to health

Thu, 01 Oct 2015 12:53:36 +0100

LONDON (Reuters) - The Welsh government said on Thursday that a dead cow on a farm in Wales had been confirmed as having bovine spongiform encephalopathy, known as mad cow disease, but said it had not entered the food chain and there was no risk to human health. (Source: Reuters: Health)



Alzheimer disease: Possible prion-like transmission of AD-like pathology in humans

Tue, 29 Sep 2015 00:00:00 +0100

Nature Reviews Neurology 11, 612 (2015). doi:10.1038/nrneurol.2015.190 Author: Hemi Malkki A small autopsy study has raised the possibility of human-to-human iatrogenic transmission of amyloid-β (Aβ) pathology, similar to that seen in Creutzfeldt–Jakob disease (CJD). The result supports the notion that Alzheimer disease (AD) pathology propagates in a prion-like fashion.John Collinge and colleagues conducted an (Source: Nature Reviews Neurology)

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ERp57 Controls PrP Levels [Neurobiology]

Fri, 25 Sep 2015 00:00:00 +0100

This study identifies ERp57 as a new modulator of PrP levels and may help with understanding the consequences of ERp57 up-regulation observed in human disease. (Source: Journal of Biological Chemistry)



Decreased regional cerebral blood flow in the bilateral thalami and medulla oblongata determined by an easy Z-score (eZIS) analysis of Tc-ECD-SPECT images in a case of MM2-thalamic-type sporadic Creutzfeldt-Jakob disease

Tue, 22 Sep 2015 00:00:00 +0100

We report a case of autopsy-verified MM2-thalamic-type sporadic Creutzfeldt-Jakob disease (sCJD) in a 46-year-old patient with a 16-month history of abnormal behavior, progressive dementia, insomnia, and speech disturbances without family history. Neurological examination revealed progressive dementia, frontal signs, insomnia, speech disturbance, gait disturbance and bilaterally exaggerated tendon reflexes. Both brain MRI and cerebrospinal fluid examinations, including 14–3–3 protein, yielded normal results. (Source: Journal of the Neurological Sciences)



Creutzfeldt-Jakob dementia

Sat, 19 Sep 2015 01:50:59 +0100

AbstractThe authors report the case of a 55-year-old female, hypertensive, smoker patient presenting with dysphonia, dysphagia and persistent dry cough. Laryngoscopy diagnosed left vocal cord paralysis. Computed tomography demonstrated saccular aneurysm of the inferior wall of the aortic arch, stretching the left recurrent laryngeal nerve, a finding compatible with Ortner’s syndrome.ResumoRelatamos um caso de paciente do sexo feminino, 55 anos, hipertensa, tabagista, apresentando disfonia, disfagia e tosse seca persistente. À videolaringoscopia foi observada paralisia da prega vocal esquerda. A tomografia computadorizada mostrou aneurisma sacular da parede inferior do arco aórtico, no local de deflexão do nervo laríngeo recorrente, permitindo diagnosticar a síndrome de Ortner. (Sour...



[A Case of Sporadic Creutzfeldt-Jakob Disease That Developed With Psychiatric Symptoms].

Tue, 15 Sep 2015 22:32:03 +0100

Authors: Özkan A, Aydın Cantürk İ, Candan F, Işık N, Özışık Karaman HI Abstract Creutzfeldt-Jakob disease (CJD) is a fairly rare prion sickness characterized by rapidly progressive dementia and neuropsychiatric symptoms. The diversity of clinical characteristics of the disease causes difficulties during diagnosis. The first finding of the disease might be psychiatric symptoms. The male patient who was diagnosed with CJD after dementia, ataxia, and myoclonus developed rapidly following psychiatric symptoms, was presented in order to draw attention to the onset with psychiatric symptoms in CJD. PMID: 26364176 [PubMed - in process] (Source: Turkish Journal of Psychiatry)



Assessing Milk from CWD-Lactating Deer for Infectious Prions

Sun, 13 Sep 2015 00:00:00 +0100

Publication date: May–July 2015 Source:New Horizons in Translational Medicine, Volume 2, Issues 4–5 Author(s): Erin McNulty, Jeanette Hayes-Klug, Kelly Anderson, Amy Nalls, Candace Mathiason Transmissible spongiform encephalopathies (TSEs), or prions, cause a fatal neurodegenerative disease affecting mammals including bovine spongiform encephalopathy (BSE) in cattle, scrapie in sheep, variant Creutzfeldt-Jakob disease in humans and chronic wasting disease (CWD) in deer, elk and moose. CWD, the only prion disease to infect a native free-ranging population, has now been detected in 22 American states, 2 Canadian provinces and South Korea. While horizontal transmission is credited for much of the spread of CWD, few studies have monitored the potential for vertical/maternal transmis...

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The Canadian Management of Bovine Spongiform Encephalopathy in Historical and Scientific Perspective, 1990-2014.

Fri, 11 Sep 2015 00:00:00 +0100

Authors: Quimby AE, Shamy MC Abstract On February 11, 2015, the Canadian Food Inspection Agency announced that a cow born and raised in Alberta had tested positive for bovine spongiform encephalopathy (BSE), commonly known as mad cow disease. BSE is a prion disease of cattle that, when transmitted to humans, produces a fatal neurodegenerative disease known as variant Creutzfeldt-Jakob disease. We believe that this latest case of BSE in Canadian cattle suggests the timeliness of a review of the management of BSE in Canada from a historically and scientifically informed perspective. In this article, we ask: how did the Canadian management of BSE between 1990 and 2014 engage with the contemporary understanding of BSE's human health implications? We propose that Canadian policies large...



Alzheimer's 'seeds' found in seven CJD victims' brains

Thu, 10 Sep 2015 16:28:00 +0100

Conclusion This small study raises questions about how a group of relatively young people with CJD came to have amyloid protein deposits in their brains when they died. But it doesn't answer those questions. The theory that amyloid proteins were transferred, along with prions, through growth hormone therapy is still just that: a theory. There are other possibilities – for example, the prions could have somehow encouraged the growth of amyloid protein. That would mean people who are already infected with prions are at an increased risk of early-onset Alzheimer's disease. However, it's also important to remember no-one in the study actually developed Alzheimer's. They may have done so had they lived longer, but we don't know. As the cause of Alzheimer's remains unknown, it is also feasibl...



No evidence that Alzheimer's can be transmitted through surgery

Wed, 09 Sep 2015 12:30:00 +0100

This study has made a possible link in four cases of specific treatment with growth hormone before 1985, but it is important not to jump to conclusions about what this important, but small, research study might mean.  There has never been a proven case of transmission by neurosurgery. Modern surgical equipment in the UK is very safe and the NHS has extremely stringent procedures to make sure of this. These include using single-use instruments where possible, and developing special equipment that reduces the risk of contamination. If single-use instruments cannot be used, then there are processes in place to track the use of specialist equipment. NHS procedures have improved significantly since the 1970s and 1980s, which is when patients in this study contracted CJD. Procedures in the NH...



Neurodegeneration: Amyloid-β pathology induced in humans

Wed, 09 Sep 2015 00:00:00 +0100

Neurodegeneration: Amyloid-β pathology induced in humans Nature 525, 7568 (2015). doi:10.1038/525193a Authors: Mathias Jucker & Lary C. Walker People who died of the neurodegenerative condition Creutzfeldt-Jakob disease after treatment with cadaver-derived human growth hormone also developed some of the pathological traits of Alzheimer's disease. See Letter p.247 (Source: Nature)



Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy

Wed, 09 Sep 2015 00:00:00 +0100

Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy Nature 525, 7568 (2015). doi:10.1038/nature15369 Authors: Zane Jaunmuktane, Simon Mead, Matthew Ellis, Jonathan D. F. Wadsworth, Andrew J. Nicoll, Joanna Kenny, Francesca Launchbury, Jacqueline Linehan, Angela Richard-Loendt, A. Sarah Walker, Peter Rudge, John Collinge & Sebastian Brandner More than two hundred individuals developed Creutzfeldt–Jakob disease (CJD) worldwide as a result of treatment, typically in childhood, with human cadaveric pituitary-derived growth hormone contaminated with prions. Although such treatment ceased in 1985, iatrogenic CJD (iCJD) continues to emerge because of the prolonged incubation periods seen in human prion infections. Unexpe...

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Efficient propagation of variant Creutzfeldt-Jakob disease prion protein using the cell-protein misfolding cyclic amplification technique with samples containing plasma and heparin.

Tue, 08 Sep 2015 00:00:00 +0100

CONCLUSION: We have established a novel cell-PMCA format in the presence of plasma without any pretreatment, where vCJD prion protein was amplified at comparable levels to that found without plasma. Our data suggest the feasibility of cell-PMCA as a practical blood test for vCJD prions. PMID: 26347231 [PubMed - as supplied by publisher] (Source: Transfusion)



An inventory of concerns behind blood safety policies in five Western countries.

Sun, 30 Aug 2015 00:00:00 +0100

CONCLUSION: Blood safety policymaking involves a wide variety of competing concerns, and approaches to reconcile these considerations are themselves contested. Developing a systematic decision-making approach requires ethical reflection on, among others, reasonable costs of safety and the value of transparency in public policy. PMID: 26331441 [PubMed - as supplied by publisher] (Source: Transfusion)



Palliative Care: A Core Competency for Stroke Neurologists [Topical Reviews]

Mon, 24 Aug 2015 00:00:00 +0100

(Source: Stroke)



Prion Disease Induces Alzheimer Disease–Like Neuropathologic Changes

Fri, 21 Aug 2015 22:59:04 +0100

We examined the brains of 266 patients with prion disease (PrionD) and found that 46 patients (17%) had Alzheimer disease (AD)–like changes. To explore potential mechanistic links between PrionD and AD, we exposed human brain aggregates (BrnAggs) to a brain homogenate from a patient with sporadic Creutzfeldt-Jakob disease and found that neurons in human BrnAggs produced many β-amyloid (Aβ; Aβ42) inclusions, whereas uninfected control-exposed human BrnAggs did not. Western blot analysis of 20 pooled Creutzfeldt-Jakob disease–infected BrnAggs verified Aβ42 levels higher than those in controls. We next examined the CA1 region of the hippocampus from 14 patients with PrionD and found that 5 patients had low levels of scrapie-associated prion protein (PrPSc), many Aβ42 intraneuronal in...



Peripheral nervous system hyperexcitability in VV2 sporadic Creutzfeldt-Jakob disease

Mon, 17 Aug 2015 00:00:00 +0100

We report 2 patients with progressive cognitive decline in the setting of peripheral nerve hyperexcitability on electrodiagnostic testing. In both patients VGKC-Abs were negative, and autopsy confirmed that both had sCJD, VV2 subtype. While uncommon, it is important to consider sCJD in patients presenting with PNHS and rapidly progressive dementia. (Source: Nature Clinical Practice)

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Diagnostic Value and Safety of Brain Biopsy in Patients With Cryptogenic Neurological Disease: A Systematic Review and Meta-analysis of 831 Cases

Sat, 25 Jul 2015 01:11:04 +0100

CONCLUSION: Brain biopsy in cryptogenic neurological disease was associated with the highest diagnostic yield in patients with suspected PACNS. The greatest clinical impact was seen in children with cryptogenic neurological disease. The presence of a radiological target was associated with a higher diagnostic yield. ABBREVIATIONS: CI, confidence interval CJD, Creutzfeldt-Jakob disease CNS, central nervous system PACNS, primary angiitis of the central nervous system RCVS, reversible cerebral vasoconstriction syndrome (Source: Neurosurgery)



Role of neuroimaging in multidisciplinary approach towards Non-Alzheimer’s dementia

Fri, 24 Jul 2015 00:00:00 +0100

Abstract Dementia is defined as chronic deterioration of intellectual function and cognitive skills significant enough to interfere with the ability to perform daily activities. Recent advances in the treatment of dementia have renewed interest in the use of various neuroimaging techniques that can assist in the diagnosis and differentiation of various subtypes. Neuroimaging and computational techniques have helped the radiological community to monitor disease progression of various neurodegenerative conditions presenting with dementia, such as Alzheimer disease, frontotemporal lobe dementia (FTLD), progressive supranuclear palsy (PSP) and multisystem atrophy-cerebellar variant (MSA-C), and their response to newer therapies. Prompt identification of treatable or reversible forms o...



The Levels of Tau Isoforms Containing Exon-2 and Exon-10 Segments Increased in the Cerebrospinal Fluids of the Patients with Sporadic Creutzfeldt-Jakob Disease

Sat, 18 Jul 2015 15:08:13 +0100

Abstract The alteration of protein tau in the cerebrospinal fluid (CSF) of Creutzfeldt-Jakob disease (CJD) has been widely evaluated, possessing a significant diagnostic value for CJD. With the biotin-labeled tau-exon-specific mAbs, direct ELISA methods were established and the levels of tau isoforms containing exon-2 and exon-10 segments in CSF of the patients with various human prion diseases and in brain tissues of scrapie-infected animals were evaluated. The results showed that the levels of tau, especially containing four repeats in microtubule binding domain, were increased in the CSF samples of the patients with sporadic CJD (sCJD). Using the unlabeled (cold) mixed exon-specific mAbs, a competitive tau ELISA was conducted based on a commercial tau kit. It revealed that the ...



Characterization of a 60‐kDa Thermally Stable Antigenic Protein as a Marker for the Immunodetection of Bovine Plasma‐Derived Food Ingredients

Tue, 14 Jul 2015 00:00:00 +0100

This study sought to establish the identity of this 60‐kDa antigenic protein and consequently determine the suitability of the sELISA for detecting bovine plasma‐derived food ingredients (BPFIs), which are widely used in dietary products without explicit labeling. Results from western blot confirmed the 60‐kDa protein to be present in the plasma fraction of bovine blood. Further proteomic analyses involving 2‐dimensional gel electrophoresis (2‐D GE) and amino acid sequencing revealed the 60‐kDa protein to be bovine serum albumin (BSA). The sELISA proved capable of detecting BPFIs in all the commercial dietary supplements tested, including those that were formulated with hydrolyzed BPFIs. The assay could also detect 0.01% and 0.5% of different BPFIs in spiked raw and cooked grou...



Inherited mtDNA variations are not strong risk factors in human prion disease

Thu, 09 Jul 2015 00:00:00 +0100

Aside from variation in the prion protein gene, genetic risk factors for sporadic Creutzfeldt-Jakob disease (sCJD) remain elusive. Given emerging evidence implicating mitochondrial dysfunction in the pathogenesis of the disorders, we studied the role of inherited mitochondrial DNA (mtDNA) variation in a 2255 sporadic prion disease cases and 3768 controls. Our analysis indicates that inherited mtDNA variation does not have a major role in the risk of developing the disorder. (Source: Neurobiology of Aging)

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vCJD in the United States - Analysis of 4 Cases (P2.329)

Mon, 06 Jul 2015 00:00:00 +0100

(Source: Neurology)



Prion trials and tribulations: Finding the right tools and experimental models

Thu, 02 Jul 2015 19:12:55 +0100

Prions are fascinating, enigmatic, and might teach us not only about rare prion diseases like Creutzfeld-Jakob disease, mad cow disease, or scrapie, but also about other more common neurodgenerative diseases. Two studies report progress with novel tools and paradigms to study prion disease. (Source: ScienceDaily Headlines)



Small Molecule Disruption of A{beta}-Prion Protein Interaction [Molecular Bases of Disease]

Thu, 02 Jul 2015 00:00:00 +0100

The prion protein (PrP) has been implicated both in prion diseases such as Creutzfeldt-Jakob disease, where its monomeric cellular isoform (PrPC) is recruited into pathogenic self-propagating polymers of misfolded protein, and in Alzheimer disease, where PrPC may act as a receptor for synaptotoxic oligomeric forms of amyloid-β (Aβ). There has been considerable interest in identification of compounds that bind to PrPC, stabilizing its native fold and thereby acting as pharmacological chaperones to block prion propagation and pathogenesis. However, compounds binding PrPC could also inhibit the binding of toxic Aβ species and may have a role in treating Alzheimer disease, a highly prevalent dementia for which there are currently no disease-modifying treatments. However, the absence of a un...



Factors influencing the survival period in Japanese patients with sporadic Creutzfeldt-Jakob disease

Tue, 30 Jun 2015 00:00:00 +0100

Although Japanese cases of sporadic Creutzfeldt-Jakob disease (sCJD) generally involve longer survival periods compared to those from other countries, details regarding the factors influencing survival are unclear. To determine the influence of certain factors on survival, we retrospectively assessed 51 Japanese MM1-type sCJD patients with respect to background, clinical course, and disease management. No significant differences were found between men and women, tracheotomy and nontracheotomy patients, or patients treated in public and other types of hospitals. (Source: Journal of the Neurological Sciences)

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Solving the next step in the mystery of prions

Thu, 25 Jun 2015 18:36:50 +0100

Working towards the ultimate goal to develop therapeutics to treat diseases such as Alzheimer's, Parkinson's, ALS, and BSE (Mad Cow Disease), scientists are investigating the physical principles underlying the formation of misfolded protein aggregates. The aggregates of misfolded proteins -- proteins that clump together in the 'wrong' structure -- feature prominently in these fatal degenerative diseases. (Source: ScienceDaily Headlines)



Can Cannibalism Fight Brain Disease? Only Sort Of.

Fri, 12 Jun 2015 18:44:37 +0100

Can cannibalism fight a rare brain disease? That’s what multiple headlines have suggested this week, but don’t pick up your fork just yet. A study published Wednesday in the scientific journal Nature found that people of Papua New Guinea’s Fore tribe -- a group that formerly consumed the brains of family members at funerals -- are now resistant to a rare, degenerative brain disease called Creutzfeldt-Jakob disease (CJD). However, the reason that they developed this resistance to the disease is because their brain-eating practice led to a major outbreak of kuru -- a specific type of CJD -- in the 1950s, Reuters reports.. A Nature news release explains that CJD occurs sporadically, but it spreads if someone consumes the brain of someone who has it. The epidemic killed as much as 2 pe...



Rethinking of doxycycline therapy in Creutzfeldt-Jakob disease

Fri, 12 Jun 2015 00:00:00 +0100

Compassionate use of doxycycline, a tetracycline antibiotic, in patients with Creutzfeldt-Jakob disease (CJD) revealed an increased survival of 4–7 months as compared with historical controls, a result not confirmed by a randomised, double blind, placebo-controlled trial.1 Is then therapy with doxycycline for patients with CJD over? The report of Assar et al2 on a single patient with variably protease-sensitive prionopathy (VPSPr),3 a rare subtype form of sporadic CJD, who received 4-year treatment with doxycycline at a relatively early stage of disease, suggests it is not and encourages novel studies on the use of doxycycline in prion diseases. The patient, who carried the commonly VPSPr-associated valine homozygosity at the polymorphic codon 129 of the prion protein (PrP) gene...



A case of variably protease-sensitive prionopathy treated with doxycyclin

Fri, 12 Jun 2015 00:00:00 +0100

We present a patient with VPSPr who received doxycycline and survived for an extended period of time in an akinetic and mute state. Neuropathological examination was performed using published methods and various anti-PrP antibodies.3 Frozen tissues from selected brain regions were available for biochemical analysis. Tissues were analysed for the... (Source: Journal of Neurology, Neurosurgery and Psychiatry)



Paradoxical herniation after decompressive craniectomy provoked by lumbar puncture or ventriculoperitoneal shunting.

Fri, 12 Jun 2015 00:00:00 +0100

CONCLUSIONS Lumbar puncture and ventriculoperitoneal shunting carry substantial risk when performed in a patient after decompressive craniectomy and before cranioplasty. When the condition that prompts decompression (such as brain swelling associated with stroke or trauma) requires time to resolve, risk is associated with lumbar puncture performed ≥ 1 month after decompressive craniectomy. PMID: 26067613 [PubMed - as supplied by publisher] (Source: Journal of Neurosurgery)

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Could brain-eating cannibals provide the key to treating CJD?

Thu, 11 Jun 2015 13:00:00 +0100

This study used genetically modified mice to test whether this genetic mutation was protective against kuru and CJD. The tests showed that mice with this genetic mutation were indeed resistant to these prion diseases.The results suggest that this mutation could be responsible for the kuru resistance seen in the survivors. It is hoped this finding may eventually help to develop effective treatments for prion diseases, but much more research will be needed to get to that point. Links To The Headlines Eating brains helped Papua New Guinea tribe become disease resistant, claims research. The Daily Telegraph, June 10 2015Eating human brains helped Papua New Guinea tribe resist disease, research shows. The Guardian, June 10 2015Scientists find CJD resistance gene. BBC News, June 10 2015 Links To...



Naturally occurring genetic variation gives complete resistance in prion diseases

Thu, 11 Jun 2015 12:00:00 +0100

Researchers at the Medical Research Council's Prion Unit have identified a naturally occurring variant of the human prion protein that produces resistance to prion diseases such as... (Source: Parkinson's Disease News From Medical News Today)



Facing up to Complexity: Implications for Our Social Experiments

Wed, 10 Jun 2015 23:53:11 +0100

Abstract Biological systems are highly complex, and for this reason there is a considerable degree of uncertainty as to the consequences of making significant interventions into their workings. Since a number of new technologies are already impinging on living systems, including our bodies, many of us have become participants in large-scale “social experiments”. I will discuss biological complexity and its relevance to the technologies that brought us BSE/vCJD and the controversy over GM foods. Then I will consider some of the complexities of our social dynamics, and argue for making a shift from using the precautionary principle to employing the approach of evaluating the introduction of new technologies by conceiving of them as social experiments. (Source: Science and Engine...



Eating brains helped Papua New Guinea tribe resist disease, research shows

Wed, 10 Jun 2015 21:16:08 +0100

The cannibalistic practice helped the Fore tribe develop genetic resistance to a mad cow-like disease. This is useful for scientists studying diseases like dementiaResearch involving a former brain-eating tribe from Papua New Guinea is helping scientists better understand mad cow disease and other so-called prion conditions and may also offer insights into Parkinson’s and dementia. People of the Fore tribe, studied by scientists from Britain and Papua New Guinea, have developed genetic resistance to a mad cow-like disease called kuru, which was spread mostly by the now abandoned ritual of eating relatives’ brains at funerals. Continue reading... (Source: Guardian Unlimited Science)



Eating human brains helped Papua New Guinea tribe resist disease, research shows

Wed, 10 Jun 2015 18:14:52 +0100

The cannibalistic practice helped the Fore tribe develop genetic resistance to a mad cow-like disease. This is useful for scientists studying diseases like dementiaResearch involving a former brain-eating tribe from Papua New Guinea is helping scientists better understand mad cow disease and other so-called prion conditions and may also offer insights into Parkinson’s and dementia. People of the Fore tribe, studied by scientists from Britain and Papua New Guinea, have developed genetic resistance to a mad cow-like disease called kuru, which was spread mostly by the now abandoned ritual of eating relatives’ brains at funerals. Continue reading... (Source: Guardian Unlimited Science)

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Genetic variant gives prion disease resistance

Wed, 10 Jun 2015 17:17:20 +0100

Researchers at the Medical Research Council’s Prion Unit have identified a naturally occurring variant of the human prion protein that produces resistance to prion diseases such as Creutzfeldt-Jakob disease (CJD). The findings, published in Nature, could give important insight into other human brain diseases that lead to dementia. (Source: Medical Research Council General News)



Scientists find mutation that protects against 'mad cow' disease after studying cannibal group

Wed, 10 Jun 2015 16:58:32 +0100

Scientists have found a genetic mutation that imparts complete protection against the human form of “mad cow” disease, which could lead to new ways of tackling similar incurable brain diseases. (Source: The Independent - Science)



Scientists find mutation that protects against 'mad cow' disease

Wed, 10 Jun 2015 16:58:32 +0100

Scientists have found a genetic mutation that imparts complete protection against the human form of “mad cow” disease, which could lead to new ways of tackling similar incurable brain diseases. (Source: The Independent - Science)



Severe, Persistent and Fatal Delirium in Psychogeriatric Patients Admitted to a Psychiatric Hospital

Wed, 10 Jun 2015 09:28:46 +0100

Conclusion: Severe, persistent and fatal delirium in patients with cognitive impairment can occur relatively early in the disease trajectory and is associated with diverse neuropathologies.Dement Geriatr Cogn Disord Extra 2015;5:253-264 (Source: Dementia and Geriatric Cognitive Disorders Extra)



A naturally occurring variant of the human prion protein completely prevents prion disease

Wed, 10 Jun 2015 00:00:00 +0100

Nature advance online publication 10 June 2015. doi:10.1038/nature14510 Authors: Emmanuel A. Asante, Michelle Smidak, Andrew Grimshaw, Richard Houghton, Andrew Tomlinson, Asif Jeelani, Tatiana Jakubcova, Shyma Hamdan, Angela Richard-Londt, Jacqueline M. Linehan, Sebastian Brandner, Michael Alpers, Jerome Whitfield, Simon Mead, Jonathan D. F. Wadsworth & John Collinge Mammalian prions, transmissible agents causing lethal neurodegenerative diseases, are composed of assemblies of misfolded cellular prion protein (PrP). A novel PrP variant, G127V, was under positive evolutionary selection during the epidemic of kuru—an acquired prion disease epidemic of the Fore population in Papua New Guinea—and appeared to provide strong protection against disease in the heterozygous state. ...

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vCJD: 30 years later

Tue, 09 Jun 2015 00:00:00 +0100

Thirty years old, a crisis took hold of British farming when it was discovered that bovine spongiform encephalopathy (BSE) in cows could leap the species barrier from cows to humans. Mark Greener examines the nature of vCJD and whether any cure is in prospect. (Source: Progress in Neurology and Psychiatry)