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creutzfeldt Jakob

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https://www.readbyqxmd.com/read/28735926/overlap-parasomnia-disorder-in-a-case-of-creutzfeldt-jakob-disease
#1
Monica Puligheddu, Patrizia Congiu, Ilaria Laccu, Michela Figorilli, Gioia Gioi, Lorenzo Polizzi, Patrizia Pisanu, F Marrosu, Federica Provini
No abstract text is available yet for this article.
August 2017: Sleep Medicine
https://www.readbyqxmd.com/read/28724625/amplification-of-misfolded-prion-proteins-in-blood-and-cerebrospinal-fluid-for-detection-of-creutzfeldt-jakob-disease
#2
Mari L DeMarco
No abstract text is available yet for this article.
July 19, 2017: Clinical Chemistry
https://www.readbyqxmd.com/read/28709448/no-added-diagnostic-value-of-non-phosphorylated-tau-fraction-p-taurel-in-csf-as-a-biomarker-for-differential-dementia-diagnosis
#3
Joery Goossens, Maria Bjerke, Hanne Struyfs, Ellis Niemantsverdriet, Charisse Somers, Tobi Van den Bossche, Sara Van Mossevelde, Bart De Vil, Anne Sieben, Jean-Jacques Martin, Patrick Cras, Johan Goeman, Peter Paul De Deyn, Christine Van Broeckhoven, Julie van der Zee, Sebastiaan Engelborghs
BACKGROUND: The Alzheimer's disease (AD) cerebrospinal fluid (CSF) biomarkers Aβ1-42, t-tau, and p-tau181 overlap with other diseases. New tau modifications or epitopes, such as the non-phosphorylated tau fraction (p-taurel), may improve differential dementia diagnosis. The goal of this study is to investigate if p-taurel can improve the diagnostic performance of the AD CSF biomarker panel for differential dementia diagnosis. METHODS: The study population consisted of 45 AD, 45 frontotemporal lobar degeneration (FTLD), 45 dementia with Lewy bodies (DLB), and 21 Creutzfeldt-Jakob disease (CJD) patients, and 20 cognitively healthy controls...
July 14, 2017: Alzheimer's Research & Therapy
https://www.readbyqxmd.com/read/28705429/disinfection-of-tonometers-a-report-by-the-american-academy-of-ophthalmology
#4
Anna K Junk, Philip P Chen, Shan C Lin, Kouros Nouri-Mahdavi, Sunita Radhakrishnan, Kuldev Singh, Teresa C Chen
OBJECTIVE: To examine the efficacy of various disinfection methods for reusable tonometer prisms in eye care and to highlight how disinfectants can damage tonometer tips and cause subsequent patient harm. METHODS: Literature searches were conducted last in October 2016 in the PubMed and the Cochrane Library databases for original research investigations. Reviews, non-English language articles, nonophthalmology articles, surveys, and case reports were excluded. RESULTS: The searches initially yielded 64 unique citations...
July 11, 2017: Ophthalmology
https://www.readbyqxmd.com/read/28680785/a-case-report-of-probable-sporadic-creutzfeldt-jakob-disease-how-to-approach-early-diagnosis
#5
Bowei Tan, Carlos Morales Mangual, Iftekhar Mahmud, Nosakhare D Tongo, Larisa Mararenko, Arthur Kay
Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare and fatal spongiform encephalopathy characterized by rapidly progressive dementia and myoclonus. The rarity of this disease and varied initial symptoms make the early diagnosis fairly challenging. Here, we present a case initially admitted for confusion and bizarre behaviors. She had acute deterioration of mental status, akinetic mutism, and myoclonus jerks four weeks later. Cerebrospinal fluid (CSF) analysis was positive for protein 14-3-3. Brain magnetic resonance imaging (MRI) showed hyperintensities in the bilateral cortex, basal ganglia, and thalami in diffusion-weighted imaging (DWI)...
May 30, 2017: Curēus
https://www.readbyqxmd.com/read/28670273/types-and-strains-their-essential-role-in-understanding-protein-aggregation-in-neurodegenerative-diseases
#6
REVIEW
Wiebke M Wemheuer, Arne Wrede, Walter J Schulz-Schaeffer
Protein misfolding and aggregation is a key event in diseases like Alzheimer's disease (AD) or Parkinson's disease (PD) and is associated with neurodegeneration. Factors that initiate protein misfolding and the role of protein aggregation in the pathophysiology of disease pose major challenges to the neuroscientific community. Interestingly, although the accumulation of the same misfolded protein, e.g., α-synuclein is detectable in all idiopathic PD patients, the disease spectrum covers a variety of different clinical presentations and disease courses...
2017: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/28668775/towards-an-early-clinical-diagnosis-of-sporadic-cjd-vv2-ataxic-type
#7
Simone Baiardi, Anna Magherini, Sabina Capellari, Veronica Redaelli, Anna Ladogana, Marcello Rossi, Fabrizio Tagliavini, Maurizio Pocchiari, Giorgio Giaccone, Piero Parchi
INTRODUCTION: Sporadic Creutzfeldt-Jakob disease (sCJD) includes a broad spectrum of clinical-pathological subtypes, which complicates the clinical differential diagnosis with other rapidly progressive neurological syndromes. AIM: To provide a better characterisation of clinical features and results of diagnostic investigations, especially at an early disease stage, in patients with sCJDVV2, the second most common sCJD subtype. METHODS: We evaluated neurological symptoms/signs, and results of brain diffusion-weighted resonance imaging (DW-MRI), electroencephalographic recordings (EEG) and cerebrospinal fluid (CSF) biomarker studies in 120 patients with a definite (n=93) or probable (n=27) diagnosis of sCJDVV2...
July 1, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28664643/a-sporadic-case-of-creutzfeldt-jakob-disease-showing-unilateral-hemispheric-involvement-on-magnetic-resonance-imaging
#8
Kentaro Hirao, Daisuke Hirose, Raita Fukasawa, Yusuke Takata, Soichiro Shimizu, Hidekazu Kanetaka, Takahiko Umahara, Hirofumi Sakurai, Haruo Hanyu
No abstract text is available yet for this article.
June 30, 2017: Psychogeriatrics: the Official Journal of the Japanese Psychogeriatric Society
https://www.readbyqxmd.com/read/28653943/a-patient-with-a-typical-presentation-of-wernicke-encephalopathy-was-found-to-have-sporadic-creutzfeldt-jakob-disease
#9
K Goossens, R L van Bruchem-Visser
Creutzfeldt-Jakob disease (CJD) has a significant degree of clinical heterogeneity that is especially found in the features at onset. Here we present a patient with the sporadic form of CJD mimicking Wernicke encephalopathy. We first treated him with a high dose of thiamine; however, the vitamin B1 levels proved to be normal, which ruled out Wernicke encephalopathy. Meanwhile, his clinical condition progressively worsened and he developed a rapidly progressive cognitive disorder, mutism and myoclonus of the muscles...
June 2017: Netherlands Journal of Medicine
https://www.readbyqxmd.com/read/28644254/sporadic-creutzfeldt-jakob-disease-with-unilateral-symptoms-in-the-setting-of-metastatic-renal-cell-carcinoma
#10
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
#11
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...
2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28576073/zno-nanoflower-based-sensitive-nano-biosensor-for-amyloid-detection
#12
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
#13
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
#14
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
#15
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
#16
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...
August 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
#17
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
#18
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
#19
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
#20
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
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