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https://www.readbyqxmd.com/read/27929120/neurofilaments-in-blood-and-csf-for-diagnosis-and-prediction-of-onset-in-creutzfeldt-jakob-disease
#1
Petra Steinacker, Kaj Blennow, Steffen Halbgebauer, Song Shi, Viktoria Ruf, Patrick Oeckl, Armin Giese, Jens Kuhle, Dana Slivarichova, Henrik Zetterberg, Markus Otto
While cerebrospinal fluid (CSF) biomarkers for Creutzfeldt-Jakob disease (CJD) are established and partly included in the diagnostic criteria, no blood biomarkers are available. Here, we assessed the utility of serum neurofilament light chain (NF-L) and tau protein in comparison to CSF markers (NF-L and phosphorylated NF heavy chain (pNF-H), tau, S100B, 14-3-3) and prion conversion assay (real-time quaking induced conversion (RT-QuIC)) for sporadic and genetic CJD. Importantly, a Gerstmann-Sträussler-Scheinker mutation carrier in the asymptomatic phase and at disease onset was included as well...
December 8, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27925304/dura-mater-graft-associated-creutzfeldt-jakob-disease-with-30-year-incubation-period
#2
Masahiro Shijo, Hiroyuki Honda, Sachiko Koyama, Koji Ishitsuka, Koichiro Maeda, Junya Kuroda, Mitsugu Tanii, Takanari Kitazono, Toru Iwaki
Over 60% of all patients with dura mater graft-associated Creutzfeldt-Jakob disease (dCJD) have been diagnosed in Japan. The incubation period has ranged from 1 to 30 years and the age at onset from 15 to 80 years. Here, we report a 77-year-old male Japanese autopsied dCJD case with the longest incubation period so far in Japan. He received a cadaveric dural graft at the right cranial convexity following a craniotomy for meningioma at the age of 46. At 30 years post-dural graft placement, disorientation was observed as an initial symptom of dCJD...
December 7, 2016: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/27905341/heidenhain-variant-of-creutzfeldt-jakob-disease-in-a-patient-who-had-bovine-bioprosthetic-valve-implantation
#3
Jehard Hashoul, Waleed Saliba, Irina Bloch, Haneen Jabaly-Habib
Creutzfeldt-Jakob disease (CJD) is a rare neurodegenerative disorder characterized by rapidly progressing dementia, general neurologic deterioration, and death. When the leading symptoms are visual disturbances, it is termed as the Heidenhain variant of CJD (HvCJD). CJD was reported following prion-contaminated pericardium transplants but never after bovine bioprosthetic cardiac valve. In this case report, we describe HvCJD in a patient who had a bovine bioprosthetic cardiac valve implant. An 82-year-old-woman was referred to neuro-ophthalmology clinic for unexplained visual loss that started 1 month previously...
October 2016: Indian Journal of Ophthalmology
https://www.readbyqxmd.com/read/27886009/diagnostic-accuracy-of-a-combined-analysis-of-cerebrospinal-fluid-t-prp-t-tau-p-tau-and-a%C3%AE-42-in-the-differential-diagnosis-of-creutzfeldt-jakob-disease-from-alzheimer-s-disease-with-emphasis-on-atypical-disease-variants
#4
Samir Abu Rumeileh, Francesca Lattanzio, Michelangelo Stanzani Maserati, Romana Rizzi, Sabina Capellari, Piero Parchi
According to recent studies, the determination of cerebrospinal fluid (CSF) total tau (t-tau)/phosphorylated tau (p-tau) ratio and total prion protein (t-PrP) levels significantly improves the accuracy of the diagnosis of Alzheimer's disease (AD) in atypical cases with clinical or laboratory features mimicking Creutzfeldt-Jakob disease (CJD). However, this has neither been validated nor tested in series including atypical CJD variants. Furthermore, the added diagnostic value of amyloid-β (Aβ)42 remains unclear...
November 19, 2016: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/27876002/sporadic-creutzfeldt-jakob-disease-with-unusual-initial-presentation-as-posterior-reversible-encephalopathy-syndrome-a-case-report
#5
Edgaras Dirzius, Renata Balnyte, Vesta Steibliene, Rymante Gleizniene, Inga Gudinaviciene, Andrius Radziunas, Kestutis Petrikonis
BACKGROUND: Creutzfeldt - Jakob disease (CJD) is a rapidly progressive and fatal neurodegenerative prion disease. MRI findings are included in diagnostic criteria for probable CJD, giving a sensitivity and specificity more than 90%, but the atypical radiological presentations in the early stage of the disease could cause the diagnostic difficulties. CJD can be definitively diagnosed by histopathological confirmation, brain biopsy or at autopsy. CASE PRESENTATION: We present a case of 53-year-old woman with a history of a rapidly progressive dementia with symptoms of visual impairment, increased extrapyramidal type muscle tonus, stereotypical movements and ataxic gait resulting in the patient's death after13 months...
November 22, 2016: BMC Neurology
https://www.readbyqxmd.com/read/27871202/corticobasal-syndrome-due-to-sporadic-creutzfeldt-jakob-disease-a-review-and-neuropsychological-case-report
#6
David Andrés González, Jason R Soble
OBJECTIVE: Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive, and fatal neurodegenerative disease with neuropsychological sequelae. This study highlighted a rare presentation of CJD (e.g. corticobasal syndrome [CBS]), reviewed updated diagnostic criteria and procedures for CJD (e.g. diffusion weighted imaging [DWI], real-time quaking-induced conversion [RT-QuIC]), and discussed differential diagnoses. METHOD: Case report methodology focused on a 68-year-old, Hispanic, right-handed man with 11 years of education...
November 22, 2016: Clinical Neuropsychologist
https://www.readbyqxmd.com/read/27870938/evaluation-of-%C3%AE-synuclein-as-a-novel-cerebrospinal-fluid-biomarker-in-different-forms-of-prion-diseases
#7
Franc Llorens, Niels Kruse, Matthias Schmitz, Nadine Gotzmann, Ewa Golanska, Katrin Thüne, Orgeta Zejneli, Eirini Kanata, Tobias Knipper, Maria Cramm, Peter Lange, Saima Zafar, Beata Sikorska, Pawel P Liberski, Eva Mitrova, Daniela Varges, Christian Schmidt, Theodoros Sklaviadis, Brit Mollenhauer, Inga Zerr
INTRODUCTION: Accurate diagnosis of prion diseases and discrimination from alternative dementias gain importance in the clinical routine, but partial overlap in cerebrospinal fluid (CSF) biomarkers impedes absolute discrimination in the differential diagnostic context. METHODS: We established the clinical parameters for prion disease diagnosis for the quantification of CSF α-synuclein in patients with sporadic (n = 234) and genetic (n = 56) prion diseases, in cases with cognitive impairment or dementia (n = 278), and in the neurologic control (n = 111) group...
November 18, 2016: Alzheimer's & Dementia: the Journal of the Alzheimer's Association
https://www.readbyqxmd.com/read/27852412/-feature-of-magnetic-resonance-imaging-in-patients-with-creutzfeldt-jakob-disease
#8
H X Wang, J Liu, Y H Yang, H P Sun, A H Liu, L P Li, J Ye, Y Hou, Y Sun, H Q Dong, Y P Wang
Objective: To assess the imaging features of 43 patients diagnosed as clinically possible or probable Creutzfeldt-Jakob disease (CJD) for providing referential information and experience on applications of MRI in the clinical diagnosis and treatment of CJD. Method: All patients who were diagnosed with "suspected CJD" and hospitalized in Xuanwu Hospital from January 2013 to November 2015 were collected, and their clinical information and MRI imaging features were analyzed retrospectively. Results: Totally 62 patients with "suspected CJD" were rectruited in this study, and 43 of them were diagnosied as probable and possible CJD when they were discharged...
October 25, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/27833227/creutzfeldt-jakob-disease-phenotype-and-course-our-experience-from-a-tertiary-center
#9
Sadanandavalli Retnaswami Chandra, Thomas Gregor Issac, Mariamma Philip, Veeranna Gadad
INTRODUCTION: Prion diseases are protein conformation disorders and neither caused by viroid or virus but is a transmissible particle labeled a prion by Pruisner. Normal prion protein becomes infectious by a different folding, but the triggers are not known. Based on the characteristic brain pathology, they are grouped under spongiform encephalopathy affecting both man and animals. Estimated prevalence is one per million. Creutzfeldt-Jakob disease (CJD) registry from National Institute and Neurosciences (NIMHANS), Bengaluru, reported 69 cases in 30 years...
September 2016: Indian Journal of Psychological Medicine
https://www.readbyqxmd.com/read/27822596/investigation-of-the-prion-protein-gene-prnp-polymorphisms-in-anatolian-murrah-and-crossbred-water-buffaloes-bubalus-bubalis
#10
Yalçın Yaman, Orhan Karadağ, Cemal Ün
Bovine spongiform encephalopathy (BSE) of the cattle is the outstanding disease among other transmissible spongiform encephalopathy (TSEs). It can be transmitted from the cattle to a human and causes a new variant of the Creutzfeldt-Jakob disease (CJD). It is known that prion protein coding gene (PRNP) plays a major role in the TSE susceptibility or resistance in some species. Recent researches demonstrated that the insertion (in) and deletion (del) polymorphisms within promoter and intron 1 region of the PRNP related to BSE susceptibility in cattle...
November 8, 2016: Tropical Animal Health and Production
https://www.readbyqxmd.com/read/27815323/many-challenges-of-conducting-clinical-trials-in-cjd
#11
EDITORIAL
Brian S Appleby
No abstract text is available yet for this article.
November 4, 2016: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/27812793/uk-iatrogenic-creutzfeldt-jakob-disease-investigating-human-prion-transmission-across-genotypic-barriers-using-human-tissue-based-and-molecular-approaches
#12
Diane L Ritchie, Marcelo A Barria, Alexander H Peden, Helen M Yull, James Kirkpatrick, Peter Adlard, James W Ironside, Mark W Head
Creutzfeldt-Jakob disease (CJD) is the prototypic human prion disease that occurs most commonly in sporadic and genetic forms, but it is also transmissible and can be acquired through medical procedures, resulting in iatrogenic CJD (iCJD). The largest numbers of iCJD cases that have occurred worldwide have resulted from contaminated cadaveric pituitary-derived human growth hormone (hGH) and its use to treat primary and secondary growth hormone deficiency. We report a comprehensive, tissue-based and molecular genetic analysis of the largest series of UK hGH-iCJD cases reported to date, including in vitro kinetic molecular modelling of genotypic factors influencing prion transmission...
November 3, 2016: Acta Neuropathologica
https://www.readbyqxmd.com/read/27807198/doxycycline-in-early-cjd-a-double-blinded-randomised-phase-ii-and-observational-study
#13
Daniela Varges, Henrike Manthey, Uta Heinemann, Claudia Ponto, Matthias Schmitz, Walter J Schulz-Schaeffer, Anna Krasnianski, Maren Breithaupt, Fabian Fincke, Katharina Kramer, Tim Friede, Inga Zerr
OBJECTIVES: The main objective of the present study is to study the therapeutic efficiency of doxycycline in a double-blinded randomised phase II study in a cohort of patients with sporadic Creutzfeldt-Jakob disease (sCJD). METHODS: From the National Reference Center of TSE Surveillance in Germany, patients with probable or definite sCJD were recruited for a double-blinded randomised study with oral doxycycline (EudraCT 2006-003934-14). In addition, we analysed the data from patients with CJD who received compassionate treatment with doxycycline in a separate group...
November 2, 2016: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/27803826/a-corticobasal-syndrome-variant-of-familial-creutzfeldt-jakob-disease-with-stroke-like-onset
#14
Ján Necpál, Martin Stelzer, Silvia Koščová, Michal Patarák
Creutzfeldt-Jakob disease (CJD) is an untreatable rare human prion disease characterized by rapidly progressive dementia along with various neurological features, including myoclonus and sometimes other movement disorders. The clinical course is typically insidious and rapid, leading to an early death. In general, the most common form is sporadic CJD; however, Slovakia is typical for a high percentage of genetic cases. We present an unusual case report of a 65-year-old man with a sudden, stroke-like onset of motor aphasia with right-sided levodopa unresponsive parkinsonism, alien hand, and other characteristic features of corticobasal syndrome (CBS), with rapid deterioration and death on the 32nd day of the disease...
2016: Case Reports in Neurological Medicine
https://www.readbyqxmd.com/read/27793473/analysis-of-the-prion-protein-gene-in-multiple-system-atrophy
#15
Viorica Chelban, Andreea Manole, Lasse Pihlstrøm, Lucia Schottlaender, Stephanie Efthymiou, Emer OConnor, Wassilios G Meissner, Janice L Holton, Henry Houlden
Neurodegenerative diseases are a very diverse group of disorders but they share some common mechanisms such as abnormally misfolded proteins with prion-like propagation and aggregation. Creutzfeldt-Jakob disease (CJD) is the most prevalent prion disease in humans. In the sporadic form of CJD the only known risk factor is the codon 129 polymorphism. Recent reports suggested that α-synuclein in multiple system atrophy (MSA) has similar pathogenic mechanisms as the prion protein. Here we present 1 Italian family with MSA and prion disease...
October 3, 2016: Neurobiology of Aging
https://www.readbyqxmd.com/read/27772745/preserved-regional-cerebral-blood-flow-in-the-occipital-cortices-brainstem-and-cerebellum-of-patients-with-v180i-129m-genetic-creutzfeldt-jakob-disease-in-serial-spect-studies
#16
Yuichi Hayashi, Nobuaki Yoshikura, Akira Takekoshi, Megumi Yamada, Takahiko Asano, Akio Kimura, Katsuya Satoh, Tetsuyuki Kitamoto, Takashi Inuzuka
Creutzfeldt-Jakob disease (CJD) with a causative point mutation of valine to isoleucine at codon 180 (V180I) is one of the major types of genetic CJD (gCJD) in Japan. V180I gCJD is rarely accompanied by a family history, and its clinical characteristics include late-onset, long disease duration, and edematous cortical hyperintensity in diffusion, fluid attenuate inversion and T2-weighted MRI. We performed serial imaging with single-photon emission computed tomography (SPECT) and MRI in three V180I gCJD cases over long-term observation...
November 15, 2016: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/27726110/reelin-expression-in-creutzfeldt-jakob-disease-and-experimental-models-of-transmissible-spongiform-encephalopathies
#17
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
https://www.readbyqxmd.com/read/27699415/diagnosing-sporadic-creutzfeldt-jakob-disease-by-the-detection-of-abnormal-prion-protein-in-patient-urine
#18
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
https://www.readbyqxmd.com/read/27690734/quality-evaluation-for-the-surveillance-system-of-human-prion-diseases-in-china-based-on-the-data-from-2010-to-2016
#19
Qi Shi, Wei Zhou, Cao Chen, Chen Gao, Kang Xiao, Jing Wang, Bao-Yun Zhang, Yuan Wang, Feng Zhang, Xiao-Ping Dong
The surveillance of CJD or human prion diseases (PrDs) has been conducted for 10 y in China. To evaluate the quality of China CJD surveillance system, the collections of the clinical and epidemiological information, the sampling, the clinical examinations and laboratory tests and follow-up survey were separately analyzed based on the data from 2010 to 2015. The obtaining rates of clinical-information table, epidemiological-information table, sample inspection sheet and medical record of the referring patients from reporting units to the center of CJD surveillance maintained or reached at very high levels, being close to 100% in the past 3 y...
November 2016: Prion
https://www.readbyqxmd.com/read/27681129/absence-of-evidence-for-a-causal-link-between-bovine-spongiform-encephalopathy-strain-variant-l-bse-and-known-forms-of-sporadic-creutzfeldt-jakob-disease-in-human-prp-transgenic-mice
#20
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 the host cellular prion protein (PrP(C)) and the infecting pathological PrP assemblies (PrP(Sc)) constituting the prion...
December 1, 2016: Journal of Virology
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