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https://www.readbyqxmd.com/read/25444937/prion-and-prion-like-diseases-in-animals
#1
REVIEW
Patricia Aguilar-Calvo, Consolación García, Juan Carlos Espinosa, Olivier Andreoletti, Juan María Torres
Transmissible spongiform encephalopaties (TSEs) are fatal neurodegenerative diseases characterized by the aggregation and accumulation of the misfolded prion protein in the brain. Other proteins such as β-amyloid, tau or Serum Amyloid-A (SAA) seem to share with prions some aspects of their pathogenic mechanism; causing a variety of so called prion-like diseases in humans and/or animals such as Alzheimer's, Parkinson's, Huntington's, Type II diabetes mellitus or amyloidosis. The question remains whether these misfolding proteins have the ability to self-propagate and transmit in a similar manner to prions...
September 2, 2015: Virus Research
https://www.readbyqxmd.com/read/25302439/hypernatremic-hemorrhagic-encephalopaty-case-report-and-literature-review
#2
Lucas Sampaio Mata, Dimitri Gusmão, Antônio Raimundo Pinto de Almeida
Hypernatremia is a common electrolyte disorder in people with impaired thirst control mechanism or access to water, and may lead from minimal disorders until coma. Among the hypernatremia morbidities, central nervous system hemorrhage is uncommon and poorly studied. We report a case involving a patient admitted to the intensive care unit with reduced consciousness level, hypernatremia and head computed tomography scan showing bilateral parenchyma hemorrhage. A literature review of hypernatremia hemorrhagic encephalopathy was conducted...
September 2010: Revista Brasileira de Terapia Intensiva
https://www.readbyqxmd.com/read/25241560/-drug-induced-eosinophilic-pleural-effusion
#3
Raluca Vasilescu
The hypersensitivity reactions induced by drugs, some widely used, like central nervous system medication, can have various presentations. The lung is a frequent target for such events. We present the case of 40-year-old male patient, non-smoker, with infant encephalopaty, seizures since age of 6 with polimorphic crisis (mainly absences), with anticonvulsivant treatment since 2011 (carbamazepine, sodium valproate, levetiracetam), with no respiratory medical history. Current symptoms started two weeks before, with chest pain, dry cough...
April 2014: Pneumologia: Revista Societății Române de Pneumologie
https://www.readbyqxmd.com/read/24637819/-history-of-human-transmissible-spongioform-encephalopaties-from-kuru-to-new-variant-of-creutzfeldt-jakob-disease
#4
N A Shnaĭder
No abstract text is available yet for this article.
2014: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
https://www.readbyqxmd.com/read/23961306/l-asparaginase-fatal-toxic-encephalopathy-during-consolidation-treatment-in-an-adult-with-acute-lymphoblastic-leukemia
#5
Frantzeska Frantzeskaki, Michalis Rizos, Matilda Papathanassiou, Nikitas Nikitas, Maria Lerikou, Apostolos Armaganidis, George Dimopoulos
PATIENT: Male, 51 FINAL DIAGNOSIS: Encephalopaty toxic Symptoms: Confusion • disorientation • drowsiness • fever MEDICATION: L-asparaginase Clinical Procedure: - Specialty: Oncology. OBJECTIVE: Unknown ethiology. BACKGROUND: Novel therapies have improved survival in malignancies of lymphoid origin. This improvement, however, has been at the cost of chemotherapy-related toxicities. L-asparaginase is frequently included in combination chemotherapies for acute lymphoblastic leukemia...
2013: American Journal of Case Reports
https://www.readbyqxmd.com/read/23548012/-liver-encephalopaty
#6
Meritxell Ventura, Juan Córdoba
No abstract text is available yet for this article.
January 2013: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/23240680/structural-models-for-cu-ii-bound-to-the-fragment-92-96-of-the-human-prion-protein
#7
Rafael Grande-Aztatzi, Lina Rivillas-Acevedo, Liliana Quintanar, Alberto Vela
The prion protein (PrP(C)) binds Cu(II) in its N-terminal region, and it is associated to a group of neurodegenerative diseases termed transmissible spongiform encephalopaties (TSEs). The isoform PrP(Sc), derived from the normal PrP(C), is the pathogenic agent of TSEs. Using spectroscopic techniques (UV-vis absorption, circular dichroism, and electron paramagnetic resonance) and electronic structure calculations, we obtained a structural description for the different pH-dependent binding modes of Cu(II) to the PrP(92-96) fragment...
January 24, 2013: Journal of Physical Chemistry. B
https://www.readbyqxmd.com/read/23056934/epileptic-encephalopathies-in-adults-and-childhood
#8
Zekiye Kural, Ali Fahir Ozer
Epileptic encephalopathies are motor-mental retardations or cognitive disorders secondary to epileptic seizures or epileptiform activities. Encephalopaties due to brain damage, medications, or systemic diseases are generally not in the scope of this definition, but they may rarely accompany the condition. Appropriate differential diagnosis of epileptic seizures as well as subclinical electroencephalographic discharges are crucial for management of seizures and epileptiform discharges and relative regression of cognitive deterioration in long-term followup...
2012: Epilepsy Research and Treatment
https://www.readbyqxmd.com/read/22937002/is-prnt-a-pseudogene-identification-of-ram-prt-in-testis-and-ejaculated-spermatozoa
#9
Jorge Pimenta, Ana Domingos, Pedro Santos, Carla C Marques, Cátia Cantante, Ana Santos, João P Barbas, Maria C Baptista, António E M Horta, Aldino Viegas, Patrícia Mesquita, João Gonçalves, Carlos A Fontes, José A M Prates, Rosa M L N Pereira
A hallmark of prion diseases or transmissible spongiform encephalopaties is the conversion of the cellular prion protein (PrP(C)), expressed by the prion gene (prnp), into an abnormally folded isoform (PrP(Sc)) with amyloid-like features that causes scrapie in sheep among other diseases. prnp together with prnd (which encodes a prion-like protein designated as Doppel), and prnt (that encodes the prion protein testis specific--Prt) with sprn (shadow of prion protein gene, that encodes Shadoo or Sho) genes, constitute the "prion gene complex"...
2012: PloS One
https://www.readbyqxmd.com/read/22347503/kosmotropic-anions-promote-conversion-of-recombinant-prion-protein-into-a-prpsc-like-misfolded-form
#10
Rodrigo Diaz-Espinoza, Abhisek Mukherjee, Claudio Soto
Prions are self-propagating proteins involved in transmissible spongiform encephalopaties in mammals. An aberrant conformation with amyloid-like features of a cell surface protein, termed prion protein (PrP), is thought to be the essential component of the infectious particle, though accessory co-factor molecules such as lipids and nucleotides may be involved. The cellular co-factors and environmental conditions implicated in PrP misfolding are not completely understood. To address this issue, several studies have been done inducing misfolding of recombinant PrP (recPrP) into classical amyloid structures using partially denaturing conditions...
2012: PloS One
https://www.readbyqxmd.com/read/20179021/is-mars-system-enough-for-a-phalloides-induced-liver-failure-treatment
#11
Laurentiu Sorodoc, Catalina Lionte, Victorita Sorodoc, Ovidiu Petris, Irina Jaba
Patients with Amanita phalloides-induced liver failure (LF) have a high mortality, despite significant advances in intensive care management. Our study evaluated the effect of Molecular Absorbents Recirculating System (MARS) comparative with optimal intensive care (OIC) in adults with this condition, in the absence of liver transplantation (LT). Six consecutive patients (women, range 16-61 years) affected by A. phalloides-induced LF were treated with OIC (3 patients) and MARS (3 patients). Laboratory parameters and hepeatic encephalopaty were evaluated 15 min before and 24 hours following each MARS treatment...
October 2010: Human & Experimental Toxicology
https://www.readbyqxmd.com/read/18568718/mismatch-of-neurophysiological-findings-in-partial-recovery-of-consciousness-a-case-report
#12
Francesca Pistoia, Simona Sacco, Raffaele Palmirotta, Paolo Onorati, Antonio Carolei, Marco Sarà
AIM: Electroencephalography (EEG) and somatosensory potentials (SEP) are regarded as useful tools for exploring residual brain activity and providing information for recovery in patients with anoxic encephalopaty. However, the diagnoses of vegetative and minimally conscious states can only be made by means of repeated specific neurological examinations. In this respect, this study describes the case of a patient with mismatch of neurophysiological findings despite partial recovery to a minimally conscious state...
July 2008: Brain Injury: [BI]
https://www.readbyqxmd.com/read/17985268/a-case-of-primary-erythermalgia-wintry-hypothermia-and-encephalopathy
#13
K Takahashi, M Saitoh, H Hoshino, M Mimaki, Y Yokoyama, M Takamizawa, M Mizuguchi, Z-M Lin, Y Yang, T Igarashi
Primary erythermalgia is a rare neuropathy characterized by attacks of burning pain and redness in the extremities in response to warm stimuli. We describe here a boy with erythermalgia whose painful attacks began in infancy. We found a novel mutation of SCN9A, which is a responsible gene for primary erythermalgia in this case. In his teens, he developed wintry hypothermia with resultant neurological dysfunction and recurrent pneumonia. During the course of pneumonia, he had transient encephalopaty with a reversible lesion in the splenium of the corpus callosum...
June 2007: Neuropediatrics
https://www.readbyqxmd.com/read/17661801/eeg-with-triphasic-waves-in-borrelia-burgdorferi-meningoencephalitis
#14
B Eriksson, L Wictor
We describe a case of encephalopathy in which the clinical picture and triphasic waves in the EEG indicated a metabolic cause. However, the illness was caused by neuroborreliosis. The occurrence of triphasic waves in the EEG is a strong evidence of metabolic encephalopathy, but triphasic waves are not specific for metabolic encephalopathy. Triphasic waves have been described in a number of non-metabolic encephalopaties and structural brain lesions. To our knowledge, this is the first report of triphasic waves in Borrelia burgdorferi meningoencephalitis...
August 2007: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/17241216/myoclonic-status-in-nonprogressive-encephalopathies-study-of-29-cases
#15
COMPARATIVE STUDY
Roberto Horacio Caraballo, Ricardo Oscar Cersósimo, Alberto Espeche, Hugo Antonio Arroyo, Natalio Fejerman
PURPOSE: Myoclonic status in nonprogressive encephalopaties (MSNE) is characterized by recurrence of long-lasting myoclonic status appearing in infants and young children with nonprogressive encephalopathy. Here, we describe the electroclinical features and evolution of MSNE. METHODS: Between February 1, 1990 and July 31, 2005, 29 patients who met diagnostic criteria of MSNE were enrolled in the study at our department and have been followed up to the present time...
January 2007: Epilepsia
https://www.readbyqxmd.com/read/17192641/effect-of-acetylcholinesterase-inhibitors-on-ache-induced-prp106-126-aggregation
#16
M V Clos, M Pera, M Ratia, S Román, P Camps, D Muñoz-Torrero, L Colombo, M Salmona, A Badia
Transmissible spongiform encephalopaties are caused by an extracellular surface protein, the scrapie prion protein (PrPsc), which is an aberrant form of normal and functional cellular PrP (PrPc). The pathological hallmarks of these diseases are the accumulation and deposition of PrPsc in the form of amyloid fibrils in the central nervous system (Tateishi et al., 1988), similar to amyloid-beta (Abeta) protein in Alzheimer's disease (AD). In some patients, Abeta and prion pathology can coexist (Hainfellner et al...
2006: Journal of Molecular Neuroscience: MN
https://www.readbyqxmd.com/read/17008136/quantitative-profiling-of-the-pathological-prion-protein-allotypes-in-bank-voles-by-liquid-chromatography-mass-spectrometry
#17
C Cartoni, M E Schininà, B Maras, R Nonno, G Vaccari, M Di Bari, M Conte, A De Pascalis, S Principe, F Cardone, M Pocchiari, U Agrimi
The conversion of the cellular prion protein (PrP(C)) into a misfolded isoform (PrP(TSE)) that accumulates in the brain of affected individuals is the key feature of transmissible spongiform encephalopaties (TSEs). Susceptibility to TSEs is influenced by polymorphisms of the prion gene suggesting that the presence of certain amino acid residues may facilitate the pathological conversion. In this work, we describe a quantitative, fast and reliable HPLC-MS method that allowed to demonstrate that in the brain of 109(Met/Ile) heterozygous bank voles infected with the mouse adapted scrapie strain 139A, there are comparable amounts of PrP(TSE) with methionine or isoleucine in position 109, suggesting that in this TSE model the two allotypes have similar rates of accumulation...
April 15, 2007: Journal of Chromatography. B, Analytical Technologies in the Biomedical and Life Sciences
https://www.readbyqxmd.com/read/16310169/influence-of-heparin-and-dendrimers-on-the-aggregation-of-two-amyloid-peptides-related-to-alzheimer-s-and-prion-diseases
#18
Barbara Klajnert, Marta Cortijo-Arellano, Maria Bryszewska, Josep Cladera
Amyloid plaques composed of proteinaceous aggregates are commonly found in brains affected by Alzheimer's disease and spongiform encephalopaties. A structural homology has been recently described for the Alzheimer's peptide Abeta1-28 and the segment of the prion protein Prp185-208. In the present paper, further elements in common are reported: the aggregation processes are in both cases enhanced by the model glucosaminoglycan heparin and dendrimers can modulate the aggregation process by affecting the nucleation rate at low concentrations and the elongation rate at high concentrations...
January 13, 2006: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/16256362/prion-protein-potentiates-acetylcholine-release-at-the-neuromuscular-junction
#19
Lamberto Re, Francesca Rossini, Francesco Re, Marica Bordicchia, Alessandra Mercanti, Olga Sonia Leon Fernandez, Simone Barocci
Cellular prion protein (PrP(c)), the normal isoform of the pathogenic peptide (PrP(sc)) responsible of the transmissible spongiform encephalopaties (TSEs), is present in many neural tissues, including neuromuscular junctions (NMJ). To analyze if this protein could influence the synaptic transmission, we performed an electrophysiological approach to study the effect of cellular prion protein on a mammalian neuromuscular junction. The loose patch clamp (LPC) technique enables the study of the whole preparation including the pre- and the post-synaptic domains...
January 2006: Pharmacological Research: the Official Journal of the Italian Pharmacological Society
https://www.readbyqxmd.com/read/16255392/neuropsychological-disorders-indicative-of-postresuscitation-encephalopaty-in-rats
#20
Natalia A Gorenkova, Irina V Nazarenko, Aleksandr V Volkov, Maria S h Avruschenko, Gennady B Lapa, Georgi I Kovalev, Lidia V Molchanova
The aim of this research was to study the effect of 12-minute clinical death on innate and acquired behavior, biogenic amine concentration, and the composition and quantity of neural populations in specific brain regions of white rats. The study shows that in animals during the postresuscitation period with formal restoration of neurological status, there are changes in emotional reactivity, orientation-exploration reactions, impairment of learning and memory, decrease in exercise tolerance and pain sensitivity...
November 2005: Spanish Journal of Psychology
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