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Creutzfeldt-Jakob

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https://www.readbyqxmd.com/read/28644254/sporadic-creutzfeldt-jakob-disease-with-unilateral-symptoms-in-the-setting-of-metastatic-renal-cell-carcinoma
#1
Kyle C Rossi, Christine M Stahl, Pengfei Zhang, John W Liang, Lara V Marcuse, Fred Lublin
INTRODUCTION: Although it is not rare for magnetic resonance imaging findings in Creutzfeldt-Jakob disease to be asymmetric, unilateral clinical syndromes are uncommonly reported and may confound diagnosis. In addition, neurological paraneoplastic syndromes are not common in renal cell carcinoma, though there are cases reported, often without an offending antibody isolated. CASE REPORT: A 66-year-old man was admitted with 1 month of left-sided numbness and "loss of control" of the left arm...
July 2017: Neurologist
https://www.readbyqxmd.com/read/28598840/neuropsychological-symptoms-in-sporadic-creutzfeldt-jakob-disease-patients-in-germany
#2
Anna Krasnianski, Geeske T Bohling, Uta Heinemann, Daniela Varges, Bettina Meissner, Walter J Schulz-Schaeffer, Andreas Reif, Inga Zerr
BACKGROUND: The polymorphism at codon 129 of the prion protein gene (PRNP) and the PrPSc types 1 and 2 belong to a molecular classification of sporadic Creutzfeldt-Jakob disease (sCJD) that correlates well with the clinical and neuropathological phenotype of sCJD. OBJECTIVE: The aim of the study was to perform the first detailed evaluation of neuropsychological deficits in a large group of definite sCJD patients with known molecular subtype. METHODS: We analyzed neuropsychological symptoms in a cohort of 248 sCJD patients with known M129 V polymorphism of PRNP and prion protein type...
June 8, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28576073/zno-nanoflower-based-sensitive-nano-biosensor-for-amyloid-detection
#3
Najim Akhtar, Sanjay Kisan Metkar, Agnishwar Girigoswami, Koyeli Girigoswami
Zinc oxide (ZnO) is a semiconductor metal oxide nanoparticle with inherent optical properties. Among the different zinc oxide nanostructures, nanoflowers have greater surface area. Utilizing this property a reagentless biosensor has been developed for the detection of beta amyloids, a hallmark of neurodegenerative diseases like Alzheimer's disease, Creutzfeldt-Jakob Syndrome, insulin dependent type II diabetes etc. The poor fluorescence quantum yield and photobleaching effect of Thioflavin T (ThT) upon binding to the model insulin amyloid beta sheets in solution can be overcome by the present engineered biosensor where ThT acts as a target as well as a reporter to detect amyloids adsorbed on a solid template based on ZnO nanoflower...
September 1, 2017: Materials Science & Engineering. C, Materials for Biological Applications
https://www.readbyqxmd.com/read/28573459/cytoskeleton-associated-risk-modifiers-involved-in-early-and-rapid-progression-of-sporadic-creutzfeldt-jakob-disease
#4
Saima Zafar, Neelam Younas, Nadeem Sheikh, Waqas Tahir, Mohsin Shafiq, Matthias Schmitz, Isidre Ferrer, Olivier Andréoletti, Inga Zerr
A high priority in the prion field is to identify pre-symptomatic events and associated profile of molecular changes. In this study, we demonstrate the pre-symptomatic dysregulation of cytoskeleton assembly and its associated cofilin-1 pathway in strain and brain region-specific manners in MM1 and VV2 subtype-specific Creutzfeldt-Jakob disease at clinical and pre-clinical stage. At physiological level, PrP(C) interaction with cofilin-1 and phosphorylated form of cofilin (p-cofilin(Ser3)) was investigated in primary cultures of mouse cortex neurons (PCNs) of PrP(C) wild-type and knockout mice (PrP(-/-))...
June 1, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28568896/an-autopsied-case-of-mv2k%C3%A2-%C3%A2-c-type-sporadic-creutzfeldt-jakob-disease-presenting-with-widespread-cerebral-cortical-involvement-and-kuru-plaques
#5
Yasushi Iwasaki, Yufuko Saito, Ikuko Aiba, Atsushi Kobayashi, Maya Mimuro, Tetsuyuki Kitamoto, Mari Yoshida
MV2-type sporadic Creutzfeldt-Jakob disease (sCJD), which was previously called "Kuru-plaque variant", was gradually revealed to have a wide spectrum and has been classified into three pathological subtypes: MV2K, MV2C and MV2K + C. We herein describe the detailed clinical findings and neuropathologic observations from an autopsied MV2K + C-type Japanese sCJD case with widespread cerebral cortical pathology and Kuru plaques. In the early stages of the disease, the patient exhibited gait disturbance with ataxia and dysarthria as well as gradual appearance of cognitive dysfunction...
June 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28553554/a-case-of-sporadic-creutzfeldt-jakob-disease-presenting-as-conversion-disorder
#6
Nikhil Yegya-Raman, Rehan Aziz, Daniel Schneider, Anthony Tobia, Megan Leitch, Onyi Nwobi
Background. Creutzfeldt-Jakob disease is a rare disorder of the central nervous system. Its initial diagnosis may be obscured by its variable presentation. This case report illustrates the complexity of diagnosing this disease early in the clinical course, especially when the initial symptoms may be psychiatric. It offers a brief review of the literature and reinforces a role for consultation psychiatry services. Methods. PUBMED/MEDLINE was searched using the terms "Creutzfeldt-Jakob disease", "psychiatric symptoms", "conversion disorder", "somatic symptom disorder", "functional movement disorder", and "functional neurologic disorder"...
2017: Case Reports in Psychiatry
https://www.readbyqxmd.com/read/28544133/autoimmune-encephalitis-associated-with-voltage-gated-potassium-channels-complex-and-leucine-rich-glioma-inactivated-1-antibodies-a-national-cohort-study
#7
M Celicanin, M Blaabjerg, C Maersk-Moller, S Beniczky, L Marner, C Thomsen, F W Bach, D Kondziella, H Andersen, F Somnier, Z Illes, L H Pinborg
BACKGROUND AND PURPOSE: The aim of this study was to describe clinical and paraclinical characteristics of all Danish patients who tested positive for anti-voltage-gated potassium channels (VGKC)-complex, anti-leucine-rich glioma-inactivated 1 (LGI1) and anti-contactin-associated protein-2 antibodies in the serum/cerebrospinal fluid between 2009 and 2013 with follow-up interviews in 2015 and 2016. METHODS: We evaluated antibody status, symptoms leading to testing, course of disease, suspected diagnosis and time of admission as well as diagnosis and treatment...
May 25, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28518033/distribution-and-quantitative-estimates-of-variant-creutzfeldt-jakob-disease-prions-in-tissues-of-clinical-and-asymptomatic-patients
#8
Jean Y Douet, Caroline Lacroux, Naima Aron, Mark W Head, Séverine Lugan, Cécile Tillier, Alvina Huor, Hervé Cassard, Mark Arnold, Vincent Beringue, James W Ironside, Olivier Andréoletti
In the United-Kingdom, ≈1 of 2,000 persons could be infected with variant Creutzfeldt-Jakob disease (vCJD). Therefore, risk of transmission of vCJD by medical procedures remains a major concern for public health authorities. In this study, we used in vitro amplification of prions by protein misfolding cyclic amplification (PMCA) to estimate distribution and level of the vCJD agent in 21 tissues from 4 patients who died of clinical vCJD and from 1 asymptomatic person with vCJD. PMCA identified major levels of vCJD prions in a range of tissues, including liver, salivary gland, kidney, lung, and bone marrow...
June 2017: Emerging Infectious Diseases
https://www.readbyqxmd.com/read/28516863/sporadic-creutzfeldt-jakob-disease-in-2-plasma-product-recipients-united-kingdom
#9
Patrick Urwin, Kumar Thanigaikumar, James W Ironside, Anna Molesworth, Richard S Knight, Patricia E Hewitt, Charlotte Llewelyn, Jan Mackenzie, Robert G Will
Sporadic Creutzfeldt-Jakob disease (sCJD) has not been previously reported in patients with clotting disorders treated with fractionated plasma products. We report 2 cases of sCJD identified in the United Kingdom in patients with a history of extended treatment for clotting disorders; 1 patient had hemophilia B and the other von Willebrand disease. Both patients had been informed previously that they were at increased risk for variant CJD because of past treatment with fractionated plasma products sourced in the United Kingdom...
June 2017: Emerging Infectious Diseases
https://www.readbyqxmd.com/read/28515556/subacute-noninfective-inflammatory-encephalopathy-our-experience-and-diagnostic-problems
#10
Sadanandavalli Retnaswami Chandra, Lakshminarayanapuram Gopal Viswanathan, Dodmalur Malikarjuna Sindhu, Anupama Ramakanth Pai
INTRODUCTION: Immune dysregulation associated encephalopathies present with significant psychiatric manifestations and only a few soft neurological and general systemic features. They are generally resistant to treatment with psychiatric medications. Generalized orthostatic myoclonus and faciobrachial dystonic seizures are mistaken as Creutzfeldt-Jakob disease and subacute sclerosing panencephalitis. PATIENTS AND METHODS: Forty-two patients seen during 2010-2015 and diagnosed as noninfective encephalopathy were analyzed...
March 2017: Indian Journal of Psychological Medicine
https://www.readbyqxmd.com/read/28509623/gastrostomy-in-patients-with-prion-disease
#11
Yasushi Iwasaki, Keiko Mori, Masumi Ito, Yoshinari Kawai, Ken-Ichiro Hoshino, Yuko Kawabata, Maya Mimuro, Mari Yoshida
Patients with prion diseases can live for long periods of time in a state of akinetic mutism given appropriate management of their symptoms. To study symptom support in these cases, we performed gastrostomies on 3 patients with V180I genetic Creutzfeldt-Jakob disease (CJD) who had become akinetic and mute, and compared them to 14 other similar patients being fed by tube. In the 3 gastrostomy cases, there were no direct complications due to the gastrostomy or tube feeding, nor were there episodes of discontinuation of tube feeding or initiation of continuous drip infusion due to severe complications...
May 4, 2017: Prion
https://www.readbyqxmd.com/read/28509609/amyloid-and-fdg-pet-in-sporadic-creutzfeldt-jakob-disease-correlation-with-pathological-prion-protein-in-neuropathology
#12
Jordi A Matías-Guiu, Carmen Guerrero-Márquez, María Nieves Cabrera-Martín, Ulises Gómez-Pinedo, María Romeral, Diego Mayo, Jesús Porta-Etessam, Teresa Moreno-Ramos, José Luis Carreras, Jorge Matías-Guiu
INTRODUCTION: The role of positron emission tomography (PET) in Creutzfeldt-Jakob disease is less defined than in other neurodegenerative diseases. We studied the correlation between the uptake of (18)F-florbetaben and (18)F-fluorodeoxyglucose with pathological prion protein deposition in histopathology in a case. METHODS: A patient with 80 y old with a rapid neurological deterioration with a confirmed diagnosis of CJD was studied. PET and MRI studies were performed between 13-20 d before the death...
May 4, 2017: Prion
https://www.readbyqxmd.com/read/28509083/detection-of-csf-14-3-3-protein-in-sporadic-creutzfeldt-jakob-disease-patients-using-a-new-automated-capillary-western-assay
#13
A Fourier, A Dorey, A Perret-Liaudet, I Quadrio
Sporadic Creutzfeldt-Jakob disease (sCJD) is a fatal rapidly progressive dementia. The detection of 14-3-3 protein in cerebrospinal fluid (CSF) is included in the WHO diagnostic criteria for the pre-mortem diagnosis of CJD. The aim of this study is to assess CSF 14-3-3 protein analytical and diagnostic performances using a new automated capillary Western technology (Simple Western technology-SW). For the validation of this assay, samples from a cohort of 268 patients suspected from sCJD were analyzed: 77 sCJD (including 40 definite sCJD) and 191 non-CJD samples were tested using both SW and the current Western Blot (WB) assays...
May 16, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28491313/positron-emission-tomography-imaging-in-a-case-of-e200k-mutation-related-spongiform-encephalopathy-with-non-diagnostic-magnetic-resonance-imaging-and-cerebrospinal-fluid-testing
#14
Pravin George, Christopher R Newey, Karin P Mente, Erik P Pioro
OBJECTIVE: Creutzfeldt-Jakob disease is a rapidly progressive spongiform encephalopathy. The E200K mutation is found in a majority of genetically transmitted Creutzfeldt-Jakob disease cases. METHODS: We describe the case and associated neuroimaging of an E200K-129M gene-mutation-related fatal spongiform encephalopathy with resultant clinical insomnia and thalamic changes. RESULTS: A 46-year-old Caucasian male presented with, who was well until 2 months prior to admission, a rapidly progressive dementia followed by a change in personality with auditory and visual hallucinations...
2017: SAGE open medical case reports
https://www.readbyqxmd.com/read/28470584/myelin-basic-protein-citrullination-a-hallmark-of-central-nervous-system-demyelination-assessed-by-novel-monoclonal-antibodies-in-prion-diseases
#15
Byungki Jang, Yong-Chul Jeon, Hae-Young Shin, Yun-Jung Lee, Hyunji Kim, Yoshitaka Kondo, Akihito Ishigami, Yong-Sun Kim, Eun-Kyoung Choi
Myelin basic protein (MBP) citrullination by peptidylarginine deiminase (PAD) enzymes leads to incomplete protein-lipid bilayer interactions and vulnerability to proteolytic enzymes, resulting in disorganization of the myelin sheath in the central nervous system. Therefore, citrullinated MBP (citMBP) has been suggested as a hallmark of demyelination, but how citMBP is implicated in prion diseases remains unknown. For the first time, we developed mouse monoclonal anti-citMBP IgG1 (clones 1B8, 1H1, and 3C6) and IgM (clone 3G5) antibodies that recognize human citMBP at its R25, R122, and R130 residues and at its C-terminal region (or the corresponding sites in mouse MBP), respectively...
May 3, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28467504/correction-fatal-prion-disease-in-a-mouse-model-of-genetic-e200k-creutzfeldt-jakob-disease
#16
Yael Friedman-Levi, Zeev Meiner, Tamar Canello, Kati Frid, Gabor G Kovacs, Herbert Budka, Dana Avrahami, Ruth Gabizon
[This corrects the article DOI: 10.1371/journal.ppat.1002350.].
May 2017: PLoS Pathogens
https://www.readbyqxmd.com/read/28455699/an-in-vivo-11-c-r-pk11195-pet-and-in-vitro-pathology-study-of-microglia-activation-in-creutzfeldt-jakob-disease
#17
Leonardo Iaccarino, Rosa Maria Moresco, Luca Presotto, Orso Bugiani, Sandro Iannaccone, Giorgio Giaccone, Fabrizio Tagliavini, Daniela Perani
Microgliosis is part of the immunobiology of Creutzfeldt-Jakob disease (CJD). This is the first report using (11)C-(R)-PK11195 PET imaging in vivo to measure 18 kDa translocator protein (TSPO) expression, indexing microglia activation, in symptomatic CJD patients, followed by a postmortem neuropathology comparison. One genetic CJD (gCJD) patient, two sporadic CJD (sCJD) patients, one variant CJD (vCJD) patient (mean ± SD age, 47.50 ± 15.95 years), and nine healthy controls (mean ± SD age, 44.00 ± 11...
April 28, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28453595/-eyelid-retraction-of-neurologic-origin-report-of-three-cases
#18
Luis Cartier R, Jorge Guzmán S, Renzo Pasquali F
Eyelid retraction, has received limited attention and it has passively been interpreted as the result of an overactive levator palpebrae superioris muscle secondary to midbrain injury. However, eyelid retractions can occur in other neurological diseases, not directly related with the midbrain. We report three patients who developed eyelid retraction. One patient had a bilateral eyelid retraction, related with Creutzfeldt-Jakob disease (CJD). Another patient had a unilateral right eyelid retraction associated with a thalamic-mesencephalic infarct...
February 2017: Revista Médica de Chile
https://www.readbyqxmd.com/read/28451263/polymorphism-at-129-dictates-metastable-conformations-of-the-human-prion-protein-n-terminal-%C3%AE-sheet
#19
S Alexis Paz, Eric Vanden-Eijnden, Cameron F Abrams
We study the thermodynamic stability of the native state of the human prion protein using a new free-energy method, replica-exchange on-the-fly parameterization. This method is designed to overcome hidden-variable sampling limitations to yield nearly error-free free-energy profiles along a conformational coordinate. We confirm that all four (M129V, D178N) polymorphs have a ground-state conformation with three intact β-sheet hydrogen bonds. Additionally, they are observed to have distinct metastabilities determined by the side-chain at position 129...
February 1, 2017: Chemical Science
https://www.readbyqxmd.com/read/28449707/altered-ca-2-homeostasis-induces-calpain-cathepsin-axis-activation-in-sporadic-creutzfeldt-jakob-disease
#20
Franc Llorens, Katrin Thüne, Beata Sikorska, Matthias Schmitz, Waqas Tahir, Natalia Fernández-Borges, Maria Cramm, Nadine Gotzmann, Margarita Carmona, Nathalie Streichenberger, Uwe Michel, Saima Zafar, Anna-Lena Schuetz, Ashish Rajput, Olivier Andréoletti, Stefan Bonn, Andre Fischer, Pawel P Liberski, Juan Maria Torres, Isidre Ferrer, Inga Zerr
Sporadic Creutzfeldt-Jakob disease (sCJD) is the most prevalent form of human prion disease and it is characterized by the presence of neuronal loss, spongiform degeneration, chronic inflammation and the accumulation of misfolded and pathogenic prion protein (PrP(Sc)). The molecular mechanisms underlying these alterations are largely unknown, but the presence of intracellular neuronal calcium (Ca(2+)) overload, a general feature in models of prion diseases, is suggested to play a key role in prion pathogenesis...
April 27, 2017: Acta Neuropathologica Communications
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