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Angela C Gauthier, Joachim M Baehring
Hashimoto's encephalopathy is a rare, imprecisely defined autoimmune neurologic syndrome associated with Hashimoto's thyroiditis that normally responds to corticosteroids. Here, we describe the case of a 55-year-old woman who presented with subacute cognitive decline and ataxia. Neoplastic, paraneoplastic, infectious, and metabolic etiologies were ruled out. Anti-TPO antibody level was markedly elevated at 966U/mL. After one month of 60mg/day of oral prednisone, she felt back to baseline and her Montreal Cognitive Assessment dramatically improved...
October 12, 2016: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
Agata Mata, Laura Urrea, Silvia Vilches, Franc Llorens, Katrin Thüne, Juan-Carlos Espinosa, Olivier Andréoletti, Alejandro M Sevillano, Juan María Torres, Jesús Rodríguez Requena, Inga Zerr, Isidro Ferrer, Rosalina Gavín, José Antonio Del Río
Reelin is an extracellular glycoprotein involved in key cellular processes in developing and adult nervous system, including regulation of neuronal migration, synapse formation, and plasticity. Most of these roles are mediated by the intracellular phosphorylation of disabled-1 (Dab1), an intracellular adaptor molecule, in turn mediated by binding Reelin to its receptors. Altered expression and glycosylation patterns of Reelin in cerebrospinal and cortical extracts have been reported in Alzheimer's disease...
October 10, 2016: Molecular Neurobiology
Oren S Cohen, Joab Chapman, Amos D Korczyn, Oliver L Siaw, Naama Warman-Alaluf, Zeev Nitsan, Shmuel Appel, Esther Kahana, Hanna Rosenmann, Chen Hoffmann
Cerebrospinal fluid (CSF) tau was found to correlate with disease severity and cognitive status in E200K familial Creutzfeldt-Jakob disease (fCJD) patients. The objective of the present study was to test whether tau levels in the CSF also correlate with the disease burden as reflected by the degree of cortical involvement in DWI MRI. Forty-four consecutive E200K fCJD patients (25 males, mean age 58.6±7.5, range 48-75 years) were recruited to the study and had a CSF tau examination as well as measurements of the extent of the cortical involvement in the DWI axial MRI...
October 6, 2016: Neuroscience Letters
Mikihiro Yamazaki, Takahiro Fukuda, Atsushi Kobayashi, Hideki Takubo
No abstract text is available yet for this article.
October 2016: Brain and Nerve, Shinkei Kenkyū No Shinpo
Connie Luk, Samantha Jones, Claire Thomas, Nick C Fox, Tze H Mok, Simon Mead, John Collinge, Graham S Jackson
Importance: Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disorder associated with the accumulation of infectious abnormal prion protein through a mechanism of templated misfolding. A recent report has described the detection of abnormal prion protein in the urine of patients with variant CJD (vCJD) using protein misfolding by cyclic amplification, which was apparently absent in the more common sporadic form of CJD (sCJD). A noninvasive diagnostic test could improve early diagnosis of sCJD and, by screening donations, mitigate the potential risks of prion transmission through human urine-derived pharmaceuticals...
October 3, 2016: JAMA Neurology
Andrew G Hughson, Brent Race, Allison Kraus, Laura R Sangaré, Lori Robins, Bradley R Groveman, Eri Saijo, Katie Phillips, Luis Contreras, Virkamal Dhaliwal, Matteo Manca, Gianluigi Zanusso, Daniel Terry, Jeffrey F Williams, Byron Caughey
Hypochlorous acid (HOCl) is produced naturally by neutrophils and other cells to kill conventional microbes in vivo. Synthetic preparations containing HOCl can also be effective as microbial disinfectants. Here we have tested whether HOCl can also inactivate prions and other self-propagating protein amyloid seeds. Prions are deadly pathogens that are notoriously difficult to inactivate, and standard microbial disinfection protocols are often inadequate. Recommended treatments for prion decontamination include strongly basic (pH ≥~12) sodium hypochlorite bleach, ≥1 N sodium hydroxide, and/or prolonged autoclaving...
September 2016: PLoS Pathogens
Emilie Jaumain, Isabelle Quadrio, Laetitia Herzog, Fabienne Reine, Human Rezaei, Olivier Andréoletti, Hubert Laude, Armand Perret-Liaudet, Stéphane Haïk, Vincent Béringue
: Prions are proteinaceous pathogens responsible for subacute spongiform encephalopathies in animals and humans. The prions responsible for bovine spongiform encephalopathy (BSE) are zoonotic agents, causing variant Creutzfeldt-Jakob disease (CJD) in humans. The transfer of prions between species is limited by a species barrier, which is thought to reflect structural incompatibilities between host cellular prion protein (PrP(C)) and the infecting pathological PrP assemblies (PrP(Sc)) constituting the prion...
September 28, 2016: Journal of Virology
Michael B Coulthart, Michael D Geschwind, Shireen Qureshi, Nicolas Phielipp, Alex Demarsh, Joseph Y Abrams, Ermias Belay, Pierluigi Gambetti, Gerard H Jansen, Anthony E Lang, Lawrence B Schonberger
As of mid-2016, 231 cases of variant Creutzfeldt-Jakob disease-the human form of a prion disease of cattle, bovine spongiform encephalopathy-have been reported from 12 countries. With few exceptions, the affected individuals had histories of extended residence in the UK or other Western European countries during the period (1980-96) of maximum global risk for human exposure to bovine spongiform encephalopathy. However, the possibility remains that other geographic foci of human infection exist, identification of which may help to foreshadow the future of the epidemic...
October 2016: Brain: a Journal of Neurology
Silvia Koscova, Dana Zakova Slivarichova, Ivana Tomeckova, Katarina Melicherova, Martin Stelzer, Alzbeta Janakova, Dana Kosorinova, Girma Belay, Eva Mitrova
Creutzfeldt-Jakob disease is a rare, but rapidly progressive, up to now untreatable and fatal neurodegenerative disorder. Clinical diagnosis of Creutzfeldt-Jakob disease (CJD) is difficult; however, it can be facilitated by suitable biomarkers. Aim of the present study is to compare levels of cerebrospinal fluid biomarkers (total tau protein, phosphorylated-tau protein, protein 14-3-3 and amyloid beta) in Slovak population of CJD suspect cases, retrospectively in over a 10-year period. One thousand three hundred sixty-four CSF samples from patients with suspect CJD, forming a homogenous group in terms of geographical as well as of equal transport conditions, storage and laboratory processing, were analysed...
September 24, 2016: Molecular Neurobiology
Oriol Grau-Rivera, Anna Calvo, Núria Bargalló, Gemma C Monté, Carlos Nos, Albert Lladó, José Luis Molinuevo, Ellen Gelpi, Raquel Sánchez-Valle
BACKGROUND: Quantitative neuroimaging might unveil abnormalities in prion diseases that are not perceivable at visual inspection. On the other hand, scarce studies have quantified volumetric changes in prion diseases. OBJECTIVES: We aim to characterize volumetric and diffusion tensor imaging (DTI) changes in patients with prion diseases who presented with either Creutzfeldt-Jakob disease (CJD) or fatal insomnia (FI) phenotype. METHODS: Twenty patients with prion diseases- 15 with CJD and 5 with fatal insomnia (FI)- and 40 healthy controls were examined with a 3-Tesla magnetic resonance imaging scanner...
September 21, 2016: Journal of Alzheimer's Disease: JAD
Mohammad Abdur Rashid, Mahmuda Haque, Mohammed Akbar
Oxidative stress in the brain is the major cause of neurodegenerative disorders, including Alzheimer's, Parkinson's, Huntington's, and Creutzfeldt-Jakob diseases or amyotrophic lateral sclerosis. Under conditions of oxidative stress, the production of highly reactive oxygen species (ROS) overwhelms antioxidant defenses, resulting in the modification of macromolecules and their deposition in neuronal cell tissues. ROS plays an important role in neuronal cell death that they generate reactive aldehydes from membrane lipid peroxidation...
2016: Advances in Neurobiology
Gabor G Kovacs
Recent studies on iatrogenic Creutzfeldt-Jakob disease (CJD) raised concerns that one of the hallmark lesions of Alzheimer disease (AD), amyloid-β (Aβ), may be transmitted from human-to-human. The neuropathology of AD-related lesions is complex. Therefore, many aspects need to be considered in deciding on this issue. Observations of recent studies can be summarized as follows: 1) The frequency of iatrogenic CJD cases with parencyhmal and vascular Aβ deposits is statistically higher than expected; 2) The morphology and distribution of Aβ deposition may show distinct features; 3) The pituitary and the dura mater themselves may serve as potential sources of Aβ seeds; 4) Cadaveric dura mater from two examined cases shows Aβ deposition; and 5) There is a lack of evidence that the clinical phenotype of AD appears following the application of cadaveric pituitary hormone or dura mater transplantation...
September 20, 2016: Prion
Maria Gabriella Vita, Dorina Tiple, Alessandra Bizzarro, Anna Ladogana, Elisa Colaizzo, Sabina Capellari, Marcello Rossi, Piero Parchi, Carlo Masullo, Maurizio Pocchiari
We report a case of rapidly evolving neurological disease in a patient with neuropathological lesions of Creutzfeldt-Jakob disease (CJD), Lewy body dementia (LBD), chronic subcortical vascular encephalopathy and meningothelial meningioma. The coexistence of severe multiple pathologies in a single patient strengthens the need to perform accurate clinical differential diagnoses in rapidly progressive dementias.
September 16, 2016: Neuropathology: Official Journal of the Japanese Society of Neuropathology
Oren S Cohen, Joab Chapman, Amos D Korczyn, Oliver L Siaw, Naama Warman-Alaluf, Zeev Nitsan, Shmuel Appel, Esther Kahana, Hanna Rosenmann, Chen Hoffmann
The use of diffusion MRI improved the accuracy of diagnosis in Creutzfeldt-Jakob disease (CJD) and expanded our knowledge of the changes occurring in the brain during the disease. The aim of this study was to test whether in patients with E200K familial CJD (fCJD) the clinical severity correlates with the disease burden as reflected by the extent of cortical involvement in DWI MRI. Consecutive fCJD patients were examined by a neurologist who performed several tests including the CJD neurological scale (CJD-NS), MiniMental status examination (MMSE), Frontal Assessment Battery (FAB), NIH Stroke Scale (NIHSS), and the expanded disability status scale (EDSS)...
September 13, 2016: Journal of Neural Transmission
Kevin Escandón-Vargas, Andrés Zorrilla-Vaca, Raúl Heli Corral-Prado
Prion diseases are rare neurodegenerative disorders occurring worldwide and affecting both humans and animals. Herein, we present the case of a patient diagnosed with definite sporadic Creutzfeldt-Jakob disease in Cali, Colombia. Besides neurological examination, 14-3-3 and tau proteins were valuable tools supporting the diagnosis. We also present a brief perspective of the prion diseases reported in Colombia to date. Although the incidence of prion diseases is unknown in Colombia, our literature review revealed that one case of scrapie in 1981 and 29 human sporadic cases of Creutzfeldt-Jakob disease have been documented and published in our country...
2016: Biomédica: Revista del Instituto Nacional de Salud
Ali Al Balushi, Marshall W Meeks, Ghazala Hayat, Jafar Kafaie
BACKGROUND: Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive neurodegenerative disease that almost always results in death in under a year from onset of symptoms. Here, we report four cases of CJD with different clinical presentations diagnosed at our institution over a 2-year period. CASES: The first patient is an 82-year-old woman who presented with depression, cognitive decline, and word-finding difficulty over 4 weeks. The patient deteriorated neurologically to akinetic mutism and death within 6 weeks of presentation...
2016: Frontiers in Neurology
Hanae Takatsuki, Takayuki Fuse, Takehiro Nakagaki, Tsuyoshi Mori, Ban Mihara, Masaki Takao, Yasushi Iwasaki, Mari Yoshida, Shigeo Murayama, Ryuichiro Atarashi, Noriyuki Nishida, Katsuya Satoh
Human prion diseases are neurodegenerative disorders caused by abnormally folded prion proteins in the central nervous system. These proteins can be detected using the quaking-induced conversion assay. Compared with other bioassays, this assay is extremely sensitive and was used in the present study to determine prion distribution in sporadic Creutzfeldt-Jakob disease patients at autopsy. Although infectivity of the sporadic form is thought to be restricted within the central nervous system, results showed that prion-seeding activities reach 10(6)/g from a 50% seeding dose in non-neuronal tissues, suggesting that prion-seeding activity exists in non-neural organs, and we suggested that non-neural tissues of 10(6)/g SD50 did not exist the infectivity...
August 24, 2016: EBioMedicine
Ester Vázquez-Fernández, Matthijn R Vos, Pavel Afanasyev, Lino Cebey, Alejandro M Sevillano, Enric Vidal, Isaac Rosa, Ludovic Renault, Adriana Ramos, Peter J Peters, José Jesús Fernández, Marin van Heel, Howard S Young, Jesús R Requena, Holger Wille
The structure of the infectious prion protein (PrPSc), which is responsible for Creutzfeldt-Jakob disease in humans and bovine spongiform encephalopathy, has escaped all attempts at elucidation due to its insolubility and propensity to aggregate. PrPSc replicates by converting the non-infectious, cellular prion protein (PrPC) into the misfolded, infectious conformer through an unknown mechanism. PrPSc and its N-terminally truncated variant, PrP 27-30, aggregate into amorphous aggregates, 2D crystals, and amyloid fibrils...
September 2016: PLoS Pathogens
Marcos Ramírez, Andrés Gallardo, Aarón Vidal, Sebastián Cornejo, Darío Ramírez, Danilo Medinas, Gonzalo Bustamante, Renzo Pasquali, Claudio Hetz
Creutzfeldt-Jakob disease has a higher incidence in Chile than in other countries. The post mortem pathological characterization of brain tissue is necessary to reach a definitive diagnosis. We report a 73 years old man with a history compatible with of a rapidly progressive dementia, in which the first electroencephalographic study showed a pattern consistent with non-convulsive status epilepticus. Besides discarding this diagnosis, it was necessary to rule out other causes of rapidly progressive dementia such as Hashimoto encephalopathy...
June 2016: Revista Médica de Chile
Roger A Moore, Young Pyo Choi, Mark W Head, James W Ironside, Robert Faris, Diane L Ritchie, Gianluigi Zanusso, Suzette Alise Priola
Aggregated and protease-resistant mammalian prion protein (PrPSc) is the primary protein component of infectious prions. Enriched PrPSc preparations are often used to study the mechanisms underlying prion disease. However, most enrichment procedures are relatively non-specific and tend to yield significant amounts of non-PrPSc components, including various proteins that could confound functional and structural studies. It is thus important to identify these proteins and assess their potential relevance to prion pathogenesis...
September 5, 2016: Journal of Proteome Research
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