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Encephalopathies

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https://www.readbyqxmd.com/read/28437822/osmotic-demyelination-syndrome-due-to-severe-hyponatremia-mimicking-hypoxic-encephalopathy
#1
Felix Wicke, Sasha Moreitz, Stefan Weidauer
Hyponatremia and its rapid correction is a well-known cause of osmotic demyelination most commonly affecting the pons. We report on a case of severe hyponatremia likely due to psychogenic polydipsia resulting in hypotonic hyperhydration with resulting cortical laminar necrosis on initial imaging, mimicking hypoxic brain damage. Pontine myelinolysis became apparent on follow-up imaging, illustrating the diagnostic challenges of extrapontine manifestations of severe hyponatremia.
April 2017: Fortschritte der Neurologie-Psychiatrie
https://www.readbyqxmd.com/read/28436817/waveform-window-38-eeg-stages-of-neonatal-hypoxic-ischemic-encephalopathy-from-background-suppression-to-resolution-of-neonatal-seizures
#2
Yuliya Lyutyy, Diana Sieciechowicz, Ahsan Nv Moosa, Elia M Pestana Knight
No abstract text is available yet for this article.
2017: Neurodiagnostic Journal
https://www.readbyqxmd.com/read/28436815/moyamoya-in-a-patient-with-fires-a-first-case-report
#3
Taylor Kaufman, Andrew White
Febrile infection-related epilepsy syndrome (FIRES) is a form of epileptic encephalopathy with severe refractory epilepsy that presents in previously healthy, school-aged children after significant febrile illness with concomitant rise in body temperature. Suspected causes include genetic or acquired channelopathies, as well as mitochondrial disturbances. In FIRES, the EEG shows diffuse slowing, generalized, and/or multifocal discharges. Seizures are present and resistant to treatment. Moyamoya angiopathy (MMA) is characterized by progressive stenosis of cerebral arteries and subsequent development of a network of collateral circulation that is prone to rupture...
2017: Neurodiagnostic Journal
https://www.readbyqxmd.com/read/28436001/gait-instability-in-valproate-treated-patients-call-to-measure-ammonia-levels
#4
S Kipervasser, C E Elger, A D Korczyn, R D Nass, C M Quesada, M Y Neufeld
OBJECTIVE: Hyperammonemia induced by valproate (VPA) treatment may lead to several neurological and systemic symptoms as well as to seizure exacerbation. Gait instability and recurrent falls are rarely mentioned as symptoms, especially not as predominant ones. METHODS: We report five adult patients with frontal lobe epilepsy (FLE) who were treated with VPA and in whom a primary adverse effect was unstable gait and falls. RESULTS: There were four males and one female patients with FLE, 25-42-year-old, three following epilepsy surgery...
April 23, 2017: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/28435905/transurethral-resection-of-the-prostate-bladder-explosion-and-hyponatremic-encephalopathy-a-rare-case-report-of-malpractice
#5
Giuseppe Vacchiano, Aldo Rocca, Rita Compagna, Anna Ginevra Immacolata Zamboli, Vera Cirillo, Lorenza Di Domenico, Veronica Di Nardo, Giuseppe Servillo, Bruno Amato
We present an original case report of a bladder explosion during a TURP intervention for benign prostatic hypertrophy, that was brought on by the absorption of about 5 liters of glycine 1.5% and then onset of a severe hyponatremia. The quick and inappropriate correction of this electrolyte imbalance led the onset of encephalopathy and the death of the patient. The authors discuss the pathogenesis of these uncommon diseases and, considering the most recent Italian Legislation, they highlight the importance to respect good clinical practice standards and guidelines to ensure the most appropriate treatments for the patient and remove any assumptions of medical liability...
January 2017: Open Medicine (Warsaw, Poland)
https://www.readbyqxmd.com/read/28435274/hashimoto-encephalopathy-with-high-plasma-monoamine-metabolite-levels-a-case-report
#6
Sho Horikoshi, Itaru Miura, Yasuto Kunii, Satoko Asano, Keiko Kanno-Nozaki, Hirobumi Mashiko, Hirooki Yabe
Hashimoto encephalopathy (HE) is believed to be an immune-mediated disorder associated with Hashimoto's thyroiditis. It was suggested that neuropsychiatric symptoms, the presence of antithyroid antibody, and good response to steroids were important for the diagnosis of HE. It has been reported that homovanillic acid (HVA) and 3-methoxy-4-hydroxyphenylglycol (MHPG), which are monoamine metabolites of dopamine and noradrenaline, respectively, are the possible biomarkers of neuropsychiatric diseases. We report a case of Hashimoto encephalopathy, in which we longitudinally measured the plasma levels of monoamine metabolites...
2017: Neuropsychiatric Disease and Treatment
https://www.readbyqxmd.com/read/28435017/association-of-paraoxonase-1-and-oxidative-stress-with-acute-kidney-injury-in-premature-asphyxiated-neonates
#7
Jasmina Ivanišević, Jelena Kotur-Stevuljević, Aleksandra Stefanović, Milica Miljković, Zorana Jelić-Ivanović, Biljana Pejović, Amira Peco-Antić
OBJECTIVES: Acute kidney injury (AKI) is defined as decrease in glomerular filtration rate with increase in serum creatinine (sCr). Perinatal asphyxia (PNA) may be an ethiological factor for AKI with oxidative stress also implicated. Paraoxonase 1 (PON1) has been reported to be decreased in renal diseases. The aim of our study was to evaluate paraoxonase 1 (PON1) and oxidative stress status, their change during the first hours and days of life and the ability of these parameters to discriminate neonates having AKI from those who do not have it...
April 20, 2017: Chemico-biological Interactions
https://www.readbyqxmd.com/read/28434268/ubiquitin-c-terminal-hydrolase-l1-uch-l1-as-a-therapeutic-and-diagnostic-target-in-neurodegeneration-neurotrauma-and-neuro-injuries
#8
Kevin K Wang, Zhihui Yang, George Sarkis, Isabel Torres, Vijaya Raghavan
Since its discovery as a major CNS-abundant protein 25 years ago, Ubiquitin C-terminal hydrolase-L1 (UCH-L1) has emerged as an important enzyme in regulating brain protein metabolism, by coupling to the proteasome pathway of protein degradation. Areas covered: UCH-L1 is implicated in both familial and sporadic Parkinson disease and other chronic neurodegenerative diseases. Also, UCH-L1 has been recently emerging as a biofluid-based biomarker for various forms of acute neurotrauma and CNS injury. Expert opinion: The loss of UCH-L1 activity coupled with the gain of proteinopathy function are linked to neurodegeneration such as Parkinsonism and Alzheimer's disease...
April 24, 2017: Expert Opinion on Therapeutic Targets
https://www.readbyqxmd.com/read/28434139/neurological-manifestations-of-atypical-celiac-disease-in-childhood
#9
Çiğdem Genç Sel, Erhan Aksoy, Ayşe Aksoy, Deniz Yüksel, Ferda Özbay
Various typical and atypical neurological manifestations can be seen as the initial symptoms of celiac disease (CD). We suggest that gluten toxicity is the most suspicious triggering risk factor for probable pathophysiological pathways of neurological involvement in atypical CD. The medical charts of 117 patients diagnosed with atypical CD were retrieved from a tertiary center in Ankara, Turkey. Eight patients reported as having neurologic manifestations as initiating symptoms were evaluated in detail. The initial neurological manifestations of CD in our study included atypical absence, which was reported first in this study, generalized tonic-clonic seizures, complex partial seizures, severe axial hypotonia and down phenotype, multifocal leukoencephalopathy, mild optic neuritis, attention deficit hyperactivity disorder, and short duration headaches...
April 22, 2017: Acta Neurologica Belgica
https://www.readbyqxmd.com/read/28434009/hypoxic-ischaemic-encephalopathy-and-the-blood-brain-barrier-in-neonates
#10
Wei Ling Amelia Lee, Adina T Michael-Titus, Divyen K Shah
This review aims to highlight a possible relationship between hypoxic-ischaemic encephalopathy (HIE) and the disruption of the blood-brain barrier (BBB). Inflammatory reactions perpetuate a large proportion of cerebral injury. The extent of injury noted in HIE is not only determined by the biochemical cascades that trigger the apoptosis-necrosis continuum of cell death in the brain parenchyma, but also by the breaching of the BBB by pro-inflammatory factors. We examine the changes that contribute to the breakdown of the BBB that occur during HIE at a macroscopic, cellular, and molecular level...
April 22, 2017: Developmental Neuroscience
https://www.readbyqxmd.com/read/28433953/the-ability-of-early-serial-developmental-assessment-to-predict-outcome-at-5years-following-neonatal-hypoxic-ischaemic-encephalopathy
#11
Catherine M O'Connor, C Anthony Ryan, Geraldine B Boylan, Deirdre M Murray
BACKGROUND: Neurodevelopmental difficulties in children following hypoxic-ischaemic encephalopathy (HIE) may not emerge until school age. AIMS: To evaluate the value and stability of early serial developmental assessments in predicting long-term outcome. STUDY DESIGN: Prospective study of infants with neonatal HIE and early continuous EEG at birth. SUBJECTS: Term infants with HIE were recruited at birth. Development was measured at 6, 12 and 24months using the Revised Griffiths' Scales (GMDS-R)...
April 20, 2017: Early Human Development
https://www.readbyqxmd.com/read/28433104/wilson-disease-symptomatic-liver-therapy
#12
Jan Pfeiffenberger, Karl-Heinz Weiss, Wolfgang Stremmel
Wilson disease leads to symptomatic impairment of liver function or liver cirrhosis. Strict adherence to decoppering agents is essential in these patients. Secondary prevention of additional hepatic damage by avoidance of other toxic substances (e.g., alcohol, drugs) and sufficient calorie intake is recommended. Routine examinations in cirrhotic patients include screening for signs of portal hypertension (esophagus varices), development of ascites, and hepatic encephalopathy. Where varices are present, primary or secondary preventive interventions may include treatment with nonselective beta-blockers or variceal ligation, similar to the approach in patients with liver cirrhosis due to other etiologies...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28431597/cefepime-induced-encephalopathy-in-end-stage-renal-disease-patients
#13
Ryo Nakagawa, Kenichiro Sato, Yoshikazu Uesaka, Takashi Mitsuki, Kanya Kondo, Atsushi Wake, Yoshifumi Ubara, Mami Kanzaki
OBJECTIVES: Impaired renal function is a risk factor for cefepime (CFPM)-induced encephalopathy (CFPMIE) in patients treated with CFPM; dose-titration to renal function is recommended to prevent CFPMIE. However, available evidence on the incidence of CFPMIE or preventive efficacy of dose adjustment against CFPMIE in end-stage renal disease (ESRD) patients is limited. METHODS: Single-centre, retrospective observational study. We reviewed consecutive in-hospital adult patients treated with adjusted-dose of CFPM in the period between September 2012 and September 2016, and assessed the CFPMIE in ESRD patients treated with adjusted-dose of CFPM...
May 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28430846/a-case-of-bariatric-surgery-related-wernicke-korsakoff-syndrome-with-persisting-anterograde-amnesia
#14
Philip Gerard Gasquoine
Objective: To describe the theoretical and clinical implications of the neuropsychological evaluation of a case of bariatric surgery-related Wernicke-Korsakoff syndrome. Method: The patient was a 37-year old, female, bilingual, bachelor's degree educated, Mexican American public relations consultant without preexisting psychiatric, neurological, or substance abuse history. Recovery from laparoscopic sleeve gastrectomy surgery for morbid obesity was complicated by intraabdominal abscess, multibacterial infection, and prolonged nausea and vomiting...
April 20, 2017: Archives of Clinical Neuropsychology: the Official Journal of the National Academy of Neuropsychologists
https://www.readbyqxmd.com/read/28430751/rhabdomyolysis-complicating-typhoid-fever-in-a-child-and-review-of-the-literature
#15
Peter James Snelling, Paul Moriarty, Vikram L Vaska, David Levitt, Clare Nourse
Typhoid fever is an important cause of morbidity and mortality in the developing world, particularly in children, but is infrequently observed in the developed world and can occur in patients without a significant travel history. Rhabdomyolysis as a complication has rarely been reported, and never in a child. A child with Salmonella enterica serovar typhi septicemia, complicated by rhabdomyolysis, encephalopathy and pancreatitis is described and all 15 reported cases to date are summarized.
April 19, 2017: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28430674/continuous-eeg-in-pediatric-critical-care-yield-and-efficiency-of-seizure-detection
#16
Arnold J Sansevere, Elizabeth D Duncan, Mark H Libenson, Tobias Loddenkemper, Phillip L Pearl, Robert C Tasker
PURPOSE: Our goal was to define the duration of continuous EEG (cEEG) monitoring needed to adequately capture electrographic seizures and EEG status epilepticus in the pediatric intensive care unit using clinical and background EEG features. METHODS: Retrospective study of patients aged 1 month to 21 years admitted to a tertiary pediatric intensive care unit and undergoing cEEG (>3 hours). Clinical data collected included admission diagnosis, EEG background features, and time variables including time to first seizure after initiation of cEEG...
April 20, 2017: Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society
https://www.readbyqxmd.com/read/28430290/frequency-and-topography-of-small-cerebrovascular-lesions-in-vascular-and-in-mixed-dementia-a-post-mortem-7-tesla-magnetic-resonance-imaging-study-with-neuropathological-correlates
#17
Jacques De Reuck, Florent Auger, Nicolas Durieux, Vincent Deramecourt, Claude-Alain Maurage, Charlotte Cordonnier, Florence Pasquier, Didier Leys, Regis Bordet
<i><b>Introduction</b>: Mixed dementia (MixD) refers to a combination of definite Alzheimer's disease (AD) and vascular encephalopathy. The existence of a "pure" type of vascular dementia (VaD) is controversial. There is a need to find magnetic resonance imaging (MRI) characteristics allowing the distinction between VaD and MixD. The present post-mortem 7.0-tesla MRI compares the frequency or severity and the topography of the small cerebrovascular lesions in brains of patients with VaD and with MixD...
2017: Folia Neuropathologica
https://www.readbyqxmd.com/read/28429419/semi-synthesis-of-murine-prion-protein-by-native-chemical-ligation-and-chemical-activation-for-preparation-of-polypeptide-%C3%AE-thioester
#18
Lei Shi, Huai Chen, Si-Yu Zhang, Ting-Ting Chu, Yu-Fen Zhao, Yong-Xiang Chen, Yan-Mei Li
Prions are suspected as pathogen of the fatal transmissible spongiform encephalopathies. Strategies to access homogenous prion protein (PrP) are required to fully comprehend the molecular mechanism of prion diseases. However, the polypeptide fragments from PrP show a high tendency to form aggregates, which is a gigantic obstacle of protein synthesis and purification. In this study, murine prion sequence 90 to 230 that is the core three-dimensional structure domain was constructed from three segments murine PrP (mPrP)(90-177), mPrP(178-212), and mPrP(213-230) by combining protein expression, chemical synthesis and chemical ligation...
April 21, 2017: Journal of Peptide Science: An Official Publication of the European Peptide Society
https://www.readbyqxmd.com/read/28428755/pharmacological-analysis-of-the-anti-epileptic-mechanisms-of-fenfluramine-in-scn1a-mutant-zebrafish
#19
Jo Sourbron, Ilse Smolders, Peter de Witte, Lieven Lagae
Dravet syndrome (DS) is a genetic encephalopathy that is characterized by severe seizures and prominent co-morbidities (e.g., physical, intellectual disabilities). More than 85% of the DS patients carry an SCN1A mutation (sodium channel, voltage gated, type I alpha subunit). Although numerous anti-epileptic drugs have entered the market since 1990, these drugs often fail to adequately control seizures in DS patients. Nonetheless, current clinical data shows significant seizure reduction in DS patients treated with the serotonergic (5-hydroxytryptamine, 5-HT) drug fenfluramine (FA)...
2017: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/28428183/risk-and-burden-of-adverse-intrapartum-related-outcomes-associated-with-non-cephalic-and-multiple-birth-in-rural-nepal-a-prospective-cohort-study
#20
Naoko Kozuki, Joanne Katz, Subarna K Khatry, James M Tielsch, Steven C LeClerq, Luke C Mullany
OBJECTIVES: Intrapartum-related complications are the second leading cause of neonatal death worldwide. We estimate the community-level risk and burden of intrapartum-related fetal/neonatal mortality and morbidity associated with non-cephalic and multiple birth in rural Sarlahi District, Nepal. DESIGN: Community-based prospective cohort study. SETTING: Rural Sarlahi District, Nepal. PARTICIPANTS: Pregnant women residing in the study area...
April 20, 2017: BMJ Open
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