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Encephalopathies

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https://www.readbyqxmd.com/read/28655027/zolpidem-for-the-treatment-of-neurologic-disorders-a-systematic-review
#1
Martin N Bomalaski, Edward S Claflin, Whitney Townsend, Mark D Peterson
Importance: Given its selective action on the ω1 subtype of the γ-aminobutyric acid A receptor, zolpidem tartrate presents a potential treatment mechanism for other neurologic disorders. Objective: To synthesize studies that used zolpidem to treat neurologic disorders. Evidence Review: Eligibility criteria included any published English-language article that examined the use of zolpidem for noninsomnia neurologic disorders in humans for all dates up to March 20, 2015...
June 26, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28654561/influenza-associated-encephalitis-encephalopathy-identified-by-the-australian-childhood-encephalitis-study-2013-2015
#2
Philip N Britton, Russell C Dale, Christopher C Blyth, Kristine Macartney, Nigel Crawford, Helen Marshall, Julia Clark, Elizabeth Elliott, Richard I Webster, Allen C Cheng, Robert Booy, Cheryl A Jones
BACKGROUND: Influenza associated encephalitis/encephalopathy (IAE) is an important cause of acute encephalitis syndrome in children. IAE includes a series of clinico-radiologic syndromes or acute encephalopathy syndromes that have been infrequently reported outside East Asia. We aimed to describe case of IAE identified by the Australian Childhood Encephalitis study. METHODS: Children ≤14 years of age with suspected encephalitis were prospectively identified in 5 hospitals in Australia...
June 24, 2017: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28653943/a-patient-with-a-typical-presentation-of-wernicke-encephalopathy-was-found-to-have-sporadic-creutzfeldt-jakob-disease
#3
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/28653647/advances-in-progressive-supranuclear-palsy-new-diagnostic-criteria-biomarkers-and-therapeutic-approaches
#4
REVIEW
Adam L Boxer, Jin-Tai Yu, Lawrence I Golbe, Irene Litvan, Anthony E Lang, Günter U Höglinger
Progressive supranuclear palsy (PSP), previously believed to be a common cause of atypical parkinsonism, is now recognised as a range of motor and behavioural syndromes that are associated with a characteristic 4-repeat tau neuropathology. New research criteria that recognise early presentations of PSP and operationalise diagnosis of the full spectrum of clinical phenotypes have been reported. The Movement Disorders Society PSP diagnostic criteria include syndromes with few or mild symptoms that are suggestive of underlying PSP pathology and could provide an opportunity for earlier therapeutic interventions in the future...
July 2017: Lancet Neurology
https://www.readbyqxmd.com/read/28652973/influenza-associated-seizures-in-healthy-adults-report-of-3-cases
#5
Aintziñe Ruisanchez-Nieva, Amaia Martinez-Arroyo, Marian Gomez-Beldarrain, Jone Bocos Portillo, Juan Carlos Garcia-Monco
To describe seizures occurring in 3 healthy adults with influenza infection. Seizures associated to influenza infection are rare in adults without encephalitis. Clinical observations of 3 healthy adult patients with influenza A and B infection and seizures. We present here 3 healthy adult patients with seizures related to influenza A or B infection without evidence encephalitis, encephalopathy or any other cause for seizures. Prognosis was excellent. Seizures can occur in healthy adults with influenza infection without evidence of encephalitis, a possibility to be borne in mind to avoid potentially harmful therapeutic and diagnostic procedures...
2017: Epilepsy & Behavior Case Reports
https://www.readbyqxmd.com/read/28652827/role-of-intrafamilial-transmission-in-high-prevalence-of-hepatitis-c-virus-in-egypt
#6
Maha Z Omar, Mohamed A Metwally, Hala M El-Feky, Inas A Ahmed, Mohamed A Ismail, Amal Idris
BACKGROUND: Egypt has the highest prevalence of hepatitis C virus (HCV) worldwide. Although an effective HCV treatment program has been adopted, it is estimated that the number of newly reported cases in Egypt is still high. Intrafamilial transmission may play a role in the high prevalence of HCV in Egypt. Identification of risk factors for the transmission of HCV may help in decreasing its prevalence and eliminating its infection. AIM: The aim of this study was to estimate the prevalence of HCV infection among the household contacts of HCV patients and identify the possible risk factors associated with intrafamilial transmission...
2017: Hepatic Medicine: Evidence and Research
https://www.readbyqxmd.com/read/28652635/-risk-factors-for-delirium-in-intensive-care-unit-br-in-liver-transplant-patients
#7
Filippo Binda, Alessandro Galazzi, Alessandra Brambilla, Ileana Adamini, Dario Laquintana
. Risk factors for delirium in intensive care unit in liver transplant patients. INTRODUCTION: Delirium is a disorder of the state of consciousness characterized by acute onset and temporal variability of cognitive abilities. It is frequent in intensive care unit (ICU) and it is associated with worst outcomes. AIM: To identify risk factors for the development of delirium in patients in intensive care unit (ICU) after orthotopic liver transplantation (OLTx). METHODS: Observational retrospective study on all the patients undergoing OLTx between January 2014 and December 2015...
April 2017: Assistenza Infermieristica e Ricerca: AIR
https://www.readbyqxmd.com/read/28649875/potential-benefits-of-zolpidem-in-disorders-of-consciousness
#8
Afsaneh Noormandi, Maryam Shahrokhi, Hossein Khalili
It has been suggested that zolpidem may arouse patients with decreased level of consciousness. Zolpidem may partially or even completely reverse abnormal cell metabolism following brain damage. In this article, available evidences regarding effects of zolpidem on disorders of consciousnesswere reviewed. Areas covered: A literature review was conducted using PubMed, Scopus, Medline, Cochrane central register of controlled trials, Cochrane database systematic reviews and Google scholar as online databases. Search Keywords were 'vegetative state','minimally conscious state','semi-comatose', 'arousal', 'zolpidem', 'wakefulness', 'awareness', and 'loss of consciousness'...
June 26, 2017: Expert Review of Clinical Pharmacology
https://www.readbyqxmd.com/read/28649562/umbilical-cord-blood-nos1-as-a-potential-biomarker-of-neonatal-encephalopathy
#9
Jun Lei, Cristina Paules, Elisabeth Nigrini, Jason M Rosenzweig, Rudhab Bahabry, Azadeh Farzin, Samuel Yang, Frances J Northington, Daniel Oros, Stephanie McKenney, Michael V Johnston, Ernest M Graham, Irina Burd
BACKGROUND: There are no definitive markers to aid in diagnosis of neonatal encephalopathy (NE). The purpose of our study was (1) to identify and evaluate the utility of neuronal nitric oxide synthase (NOS1) in umbilical cord blood as a NE biomarker and (2) to identify the source of NOS1 in umbilical cord blood. METHODS: This was a nested case-control study of neonates >35 weeks of gestation. ELISA for NOS1 in umbilical cord blood was performed. Sources of NOS1 in umbilical cord were investigated by immunohistochemistry, western blot, ELISA, and quantitative PCR...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28649556/successful-pregnancy-and-delivery-in-a-woman-with-propionic-acidemia-from-the-amish-community
#10
Jessica Scott Schwoerer, Sandra van Calcar, Gregory M Rice, James Deline
Propionic acidemia (PA) is an inborn error of protein metabolism with a variable clinical presentation ranging from neonatal encephalopathy to seemingly asymptomatic individuals who present with cardiomyopathy or sudden death. PA is recognized in the Amish population, often with an early asymptomatic course and eventual cardiac complications. Thus, Amish women with PA may reach reproductive age without clinical sequelae, but are at increased risk for metabolic decompensation during pregnancy, delivery and postpartum period...
September 2016: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/28649495/non-hyperammonaemic-valproate-encephalopathy-after-20%C3%A2-years-of-treatment
#11
Elizabeth Caruana Galizia, Jeremy D Isaacs, Hannah R Cock
Sodium valproate is a commonly used antiseizure drug with broad indications for different seizuretypes and epilepsy syndromes. Well-recognised side effects include weight gain, tremor, dizziness, and unsteadiness. Non-hyperammonaemic parkinsonism, with or without cognitive impairment, is a rare adverse effect of sodium valproate. We present the case of a sixty year-old lady with a generalized seizure disorder, treated with phenytoin, valproate, lamotrigine and clonazepam. Following withdrawal of phenytoin she developed an akinetic-rigid syndrome, with ataxia and marked cognitive impairment...
2017: Epilepsy & Behavior Case Reports
https://www.readbyqxmd.com/read/28649286/autism-spectrum-disorder-and-epileptic-encephalopathy-common-causes-many-questions
#12
REVIEW
Siddharth Srivastava, Mustafa Sahin
Epileptic encephalopathies represent a particularly severe form of epilepsy, associated with cognitive and behavioral deficits, including impaired social-communication and restricted, repetitive behaviors that are the hallmarks of autism spectrum disorder (ASD). With the advent of next-generation sequencing, the genetic landscape of epileptic encephalopathies is growing and demonstrates overlap with genes separately implicated in ASD. However, many questions remain about this connection, including whether epileptiform activity itself contributes to the development of ASD symptomatology...
2017: Journal of Neurodevelopmental Disorders
https://www.readbyqxmd.com/read/28648719/modification-to-the-rice-vannucci-perinatal-hypoxic-ischaemic-encephalopathy-model-in-the-p7-rat-improves-the-reliability-of-cerebral-infarct-development-after-48hours
#13
Adam B Edwards, Kirk W Feindel, Jane L Cross, Ryan S Anderton, Vincent W Clark, Neville W Knuckey, Bruno P Meloni
BACKGROUND: The Rice-Vannucci model of hypoxic-ischaemic encephalopathy (HIE) has been associated with a high degree of variability with respect to the development of cerebral infarction and infarct lesion volume. For this reason, we examined the occurrence of communicational blood flow within the common carotid (CCA), internal (ICA), and external (ECA) carotid arteries following CCA occlusion as a source of variability in the model. NEW METHOD: We propose a novel modification to the Rice-Vannucci model, whereby both the CCA and ECA are permanently ligated; mitigating communicational blood flow...
June 22, 2017: Journal of Neuroscience Methods
https://www.readbyqxmd.com/read/28648459/acute-liver-failure-is-associated-with-altered-cerebral-expression-profiles-of-long-non-coding-rnas
#14
Vinícius R Silva, Rodrigo Secolin, Raghu Vemuganti, Iscia Lopes-Cendes, Alan S Hazell
Hepatic encephalopathy (HE) represents a serious complication of acute liver failure (ALF) in which cerebral edema leading to brainstem herniation as a result of increased intracranial hypertension is a major consequence. Long non-coding RNAs (lncRNAs) play a significant role in coordinating gene expression, with recent studies indicating an influence in the pathogenesis of several diseases. To investigate their involvement in the cerebral pathophysiology of ALF, we profiled the expression of lncRNAs in the frontal cortex of mice at coma stage following treatment with the hepatotoxin azoxymethane...
June 22, 2017: Neuroscience Letters
https://www.readbyqxmd.com/read/28648366/-oxidative-stress-in-perinatal-asphyxia-and-hypoxic-ischaemic-encephalopathy
#15
Antonio Nuñez, Isabel Benavente, Dorotea Blanco, Héctor Boix, Fernando Cabañas, Mercedes Chaffanel, Belén Fernández-Colomer, José Ramón Fernández-Lorenzo, Begoña Loureiro, María Teresa Moral, Antonio Pavón, Inés Tofé, Eva Valverde, Máximo Vento
Birth asphyxia is one of the principal causes of early neonatal death. In survivors it may evolve to hypoxic-ischaemic encephalopathy and major long-term neurological morbidity. Prolonged and intense asphyxia will lead to energy exhaustion in tissues exclusively dependent on aerobic metabolism, such as the central nervous system. Energy deficit leads to ATP-dependent pumps blockage, with the subsequent loss of neuronal transmembrane potential. The most sensitive areas of the brain will die due to necrosis. In more resistant areas, neuronal hyper-excitability, massive entrance of ionic calcium, activation of NO-synthase, free radical generation, and alteration in mitochondrial metabolism will lead to a secondary energy failure and programmed neuronal death by means of the activation of the caspase pathways...
June 22, 2017: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
https://www.readbyqxmd.com/read/28647130/fever-induced-paroxysmal-weakness-and-encephalopathy-a-new-phenotype-of-atp1a3-mutation
#16
Sho T Yano, Kenneth Silver, Richard Young, Suzanne D DeBrosse, Roseànne S Ebel, Kathryn J Swoboda, Gyula Acsadi
BACKGROUND: We identified a group of patients with ATP1A3 mutations at residue 756 who display a new phenotype, distinct from alternating hemiplegia of childhood, rapid-onset dystonia-parkinsonism, and cerebellar ataxia, areflexia, pes cavus, optic atrophy, sensorineural hearing loss syndromes. METHODS: Four patients with c.2267G>A (R756H) mutations from two families and two patients with c.2267G>T (R756L) mutations from one family are described and compared with the previously reported patients with mutations resulting in R756H and R756C protein variants...
April 29, 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/28645740/safety-of-the-2d-3d-direct-acting-antiviral-regimen-in-hcv-induced-child-pugh-a-cirrhosis-a-pooled-analysis
#17
Fred Poordad, David R Nelson, Jordan J Feld, Michael W Fried, Heiner Wedemeyer, Lois Larsen, Daniel E Cohen, Eric Cohen, Niloufar Mobashery, Fernando Tatsch, Graham R Foster
BACKGROUND & AIMS: Chronic hepatitis C virus (HCV)-infected patients with cirrhosis are a high-priority population for treatment. To help inform the benefit-risk profile of the all-oral direct-acting antiviral (DAA) combination regimen of ombitasvir, paritaprevir, and ritonavir, with or without dasabuvir (OBV/PTV/r ± DSV) in patients with Child-Pugh A cirrhosis, we undertook a comprehensive review of AbbVie-sponsored clinical trials enrolling patients with Child-Pugh A cirrhosis. METHODS: Twelve phase II or III clinical trials of the 2-DAA regimen of OBV/PTV/r ± ribavirin (RBV) or the 3-DAA regimen of OBV/PTV/r + DSV ± RBV that included patients with Child-Pugh A cirrhosis were reviewed; patients who completed treatment by November 16, 2015 were included in a pooled, post hoc safety assessment...
June 20, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/28645153/defective-mitochondrial-rna-processing-due-to-pnpt1-variants-causes-leigh-syndrome
#18
Sanna Matilainen, Christopher J Carroll, Uwe Richter, Liliya Euro, Max Pohjanpelto, Anders Paetau, Pirjo Isohanni, Anu Suomalainen
Leigh syndrome is a severe infantile encephalopathy with an exceptionally variable genetic background. We studied the exome of a child manifesting with Leigh syndrome at one month of age and progressing to death by the age of 2.4 years, and identified novel compound heterozygous variants in PNPT1, encoding the polynucleotide phosphorylase (PNPase). Expression of the wild type PNPT1 in the subject's myoblasts functionally complemented the defects, and the pathogenicity was further supported by structural predictions and protein and RNA analyses...
June 22, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28644255/periodic-paralysis-and-encephalopathy-as-initial-manifestations-of-graves-disease-case-report-and-review-of-the-literature
#19
Theocharis Tsironis, Athanasios Tychalas, Dimitrios Kiourtidis, Jannis Kountouras, Georgia Xiromerisiou, Jobst Rudolf, Georgia Deretzi
BACKGROUND: Thyrotoxic periodic paralysis (TPP) is an uncommon complication of Graves' disease, characterized by the triad of acute hypokalemia without total body potassium deficit, episodic muscle paralysis, and thyrotoxicosis. Graves' encephalopathy is an extremely rare form of encephalopathy associated with autoimmune thyroid disease (EAATD), characterized by neuropsychiatric symptoms, increased antithyroid antibodies and cerebrospinal fluid protein concentration, nonspecific electroencephalogram abnormalities, and cortico-responsiveness...
July 2017: Neurologist
https://www.readbyqxmd.com/read/28644254/sporadic-creutzfeldt-jakob-disease-with-unilateral-symptoms-in-the-setting-of-metastatic-renal-cell-carcinoma
#20
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
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