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Dysautonomia

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https://www.readbyqxmd.com/read/28187808/diagnosis-and-management-of-acute-encephalitis
#1
J J Halperin
Encephalitis is typically viral (approximately half of diagnosed cases) or autoimmune (about a quarter) with the remainder remaining undiagnosable at this time. All require general supportive care but only a minority requires intensive care admission - in these intubation, to protect the airway or to treat status epilepticus with anesthetic drugs, may be needed. In some dysautonomia with wide blood pressure fluctuations is the principal concern. Remarkably, in addition to supportive care, specific treatment options are available for the majority - immune-modulating therapy for those with autoimmune disorders, antiviral therapy for herpes simplex 1 and 2, and varicella-zoster encephalitis...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28179209/administration-of-anabolic-steroid-during-adolescence-induces-long-term-cardiac-hypertrophy-and-increases-susceptibility-to-ischemia-reperfusion-injury-in-adult-wistar-rats
#2
Fernando de Azevedo Cruz Seara, Raiana Andrade Quintanilha Barbosa, Dahienne Ferreira de Oliveira, Diorney Luiz Souza Gran da Silva, Adriana Bastos Carvalho, Andrea Claudia Freitas Ferreira, José Hamilton Matheus Nascimento, Emerson Lopes Olivares
Chronic administration of anabolic androgenic steroids (AAS) in adult rats results in cardiac hypertrophy and increased susceptibility to myocardial ischemia/reperfusion (IR) injury. Molecular analyses demonstrated that hyperactivation of type 1 angiotensin II (AT1) receptor mediates cardiac hypertrophy induced by AAS and also induces down-regulation of myocardial ATP-sensitive potassium channel (KATP), resulting in loss of exercise-induced cardioprotection. Exposure to AAS during adolescence promoted long-term cardiovascular dysfunctions, such as dysautonomia...
February 5, 2017: Journal of Steroid Biochemistry and Molecular Biology
https://www.readbyqxmd.com/read/28167615/the-familial-dysautonomia-disease-gene-ikbkap-elp1-is-required-in-the-developing-and-adult-central-nervous-system
#3
Marta Chaverra, Lynn George, Marc Mergy, Hannah Waller, Katharine Kujawa, Connor Murnion, Ezekiel Sharples, Julian Thorne, Nathaniel Podgajny, Andrea Grindeland, Yumi Ueki, Steven Eiger, Cassie Cusick, A Michael Babcock, George A Carlson, Frances Lefcort
Hereditary sensory and autonomic neuropathies (HSANs) are a genetically and clinically diverse group of disorders defined by peripheral nervous system (PNS) dysfunction. HSAN Type III, Familial Dysautonomia (FD), results from a single base mutation in the gene IKBKAP that encodes a scaffolding unit for a multi-subunit complex Elongator. Since mutations in other Elongator subunits (ELP2-4) are associated with central nervous system (CNS) disorders, the goal of this study was to investigate a potential CNS requirement for Ikbkap/Elp1 The sensory and autonomic pathophysiology of FD is fatal, with the majority of patients dying by age 40...
February 6, 2017: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/28164144/pure-autonomic-failure-versus-prodromal-dysautonomia-in-parkinson%C3%A2-s-disease-insights-from-the-bedside
#4
Aristide Merola, Alberto J Espay, Maurizio Zibetti, Alberto Romagnolo, Michela Rosso, Simona Maule, Leonardo Lopiano
No abstract text is available yet for this article.
2017: Movement Disorders Clinical Practice
https://www.readbyqxmd.com/read/28162168/-analysis-of-clinical-characteristics-and-outcomes-of-mechanically-ventilated-patients-with-anti-n-methyl-d-aspartate-receptor-encephalitis
#5
Y Zhang, Y Y Su, D Q Gao, H Ye
Objective: To explore the clinical characteristics and long-term outcomes of mechanically ventilated patients with anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis. Methods: In this observational study, patients with anti-NMDAR encephalitis were enrolled, who were admitted into Xuanwu Hospital of Capital Medical University from Jan 2012 to Jun 2015.All patients accepted tumor screening, symptomatic therapy, and immunotherapy.Outcomes were assessed by modified Rankin Scale (mRS) after immunotherapy every 6 months, and mRS 0-2 was defined as favorable outcome...
January 17, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28161223/genetic-polymorphisms-in-the-esr1-and-vdr-genes-do-not-correlate-with-osteoporosis-in-patients-with-familial-dysautonomia
#6
David Cheishvili, Channa Maayan, Daniel M Sapozhnikov, Elad Lax, Rivka Dresner-Pollak
One of the major clinical manifestations of familial dysautonomia (FD)-a rare, neurodegenerative, autosomal-recessive disorder-is a high incidence and early onset of osteoporotic bone fractures. Early diagnosis is essential to initiate preventative therapy in at-risk patients and thus improve quality of life. However, the current lack of understanding of the complex relationship between FD and osteoporosis etiology precludes early diagnosis, and as such, accurate predictors of osteoporosis development in FD patients remain to be determined...
February 1, 2017: Journal of Clinical Densitometry
https://www.readbyqxmd.com/read/28149085/complications-in-mechanically-ventilated-patients-of-guillain-barre-syndrome-and-their-prognostic-value
#7
Archana Becket Netto, Arun B Taly, Girish B Kulkarni, G S Uma Maheshwara Rao, Shivaji Rao
INTRODUCTION: The spectrum of various complications in critically ill Guillain-Barre syndrome (GBS) and its effect on the prognosis is lacking in literature. This study aimed at enumerating the complications in such a cohort and their significance in the prognosis and mortality. MATERIALS AND METHODS: Retrospective case record analysis of all consecutive mechanically ventilated patients of GBS in neurology Intensive Care Unit (ICU) of a tertiary care institute for 10 years was done...
January 2017: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/28149069/impact-of-yoga-in-a-case-of-vocal-cord-dysfunction-with-dysautonomia
#8
Rozina Wadhwania
A 23-year-old female with a past medical history of gastroesophageal reflux disease presented with shortness of breath induced by exercise and certain odors. She reported the symptoms of autonomic dysfunction including fatigue, chest pain, lightheadedness, headaches, numbness/tingling in the arms and legs, and exercise intolerance. Vital signs were significant for orthostatic intolerance. Volume flow loop in the pulmonary function tests showed a flattening of the inspiratory portion characteristic of vocal cord dysfunction...
January 2017: International Journal of Yoga
https://www.readbyqxmd.com/read/28144147/neuroleptic-malignant-syndrome-an-easily-overlooked-neurologic-emergency
#9
REVIEW
Ramadhan Oruch, Ian F Pryme, Bernt A Engelsen, Anders Lund
Neuroleptic malignant syndrome is an unpredictable iatrogenic neurologic emergency condition, mainly arising as an idiosyncratic reaction to antipsychotic agent use. It is characterized by distinctive clinical features including a change in mental status, generalized rigidity, hyperpyrexia, and dysautonomia. It can be lethal if not diagnosed and treated properly. Mortality and morbidity attributed to this syndrome have recently declined markedly due to greater awareness, earlier diagnosis, and intensive care intervention...
2017: Neuropsychiatric Disease and Treatment
https://www.readbyqxmd.com/read/28128656/anesthetic-management-of-a-child-with-unspecified-mitochondrial-disease-in-an-outpatient-dental-setting
#10
Taylor R Gordon, Richard J Montandon
Mitochondrial disease (MD) represents a category of metabolic disorders with a wide range of symptoms across a variety of organ systems. It occurs with an incidence of greater than 1:5000 and can be difficult to specifically diagnose because of the variety of clinical presentations and multiple genomic origins. Although phenotypically variable, MD symptoms often include hypotonia, cardiac defects, dysautonomia, and metabolic dysfunction. Mitochondrial disease presents a unique challenge in terms of anesthetic management, as many anesthetic drugs suppress mitochondrial function...
2017: Anesthesia Progress
https://www.readbyqxmd.com/read/28126506/-other-causes-of-parkinsonism
#11
Christine Tranchant
Numerous causes may induce parkinsonism, and in case of Parkinsonism, l-dopa resistance, associated neurological signs, early falls, early dysautonomia or early cognitive disorders are the main red flags which should lead to exclude Parkinson's disease. Drug-induced parkinsonism and in young people, Wilson disease, should always be searched. Lower limbs parkinsonism is often due to vascular lesions. Multiple systemic atrophy, supranuclear palsy, Lewy body disease and the rarer corticobasal degeneration, each have specific clinical signs...
January 23, 2017: La Presse Médicale
https://www.readbyqxmd.com/read/28120078/brainstem-and-autonomic-nervous-system-dysfunction-a-neurosurgical-point-of-view
#12
A Martín-Gallego, L González-García, A Carrasco-Brenes, M Segura-Fernández-Nogueras, A Delgado-Babiano, A Ros-Sanjuán, L Romero-Moreno, M Domínguez-Páez, M S Dawid-Milner, M A Arráez-Sánchez
Central autonomic control nuclei and pathways are mainly integrated within the brainstem, especially in the medulla oblongata. Lesions within these structures can lead to central dysautonomia.Central autonomic control structures can be damaged by tumors, during surgery, or by other neurosurgical pathologies. These may elicit clinical or subclinical autonomic complications that can constitute a serious clinical problem.The authors present a broad review of the central autonomic nervous system, its possible dysfunctions, and the relation between neurosurgery and this "not-well-known system"...
2017: Acta Neurochirurgica. Supplement
https://www.readbyqxmd.com/read/28112424/exercise-and-the-multidisciplinary-holistic-approach-to-adolescent-dysautonomia
#13
K Armstrong, A M De Souza, P Sneddon, J E Potts, V E Claydon, S Sanatani
AIM: To determine if an 8-week strength training program as part of a multidisciplinary approach would minimize symptoms and improve quality of life in patients with dysautonomia. METHODS: Adolescents referred to a tertiary level Cardiology service from May 2014-Dec 2015 with symptoms of dysautonomia were eligible. Participants completed an exercise test and a quality of life (QoL) questionnaire (Peds QL) prior to the intervention. Participants were asked to complete exercises 5 times per week...
January 23, 2017: Acta Paediatrica
https://www.readbyqxmd.com/read/28110698/heart-rate-variability-regression-and-risk-of-sudden-unexpected-death-in-epilepsy
#14
Alessio Galli, Federico Lombardi
The exact mechanisms of sudden unexpected death in epilepsy remain elusive, despite there is consensus that SUDEP is associated with severe derangements in the autonomic control to vital functions as breathing and heart rate regulation. Heart rate variability (HRV) has been advocated as biomarker of autonomic control to the heart. Cardiac dysautonomia has been found in diseases where other branches of the autonomous nervous system are damaged, as Parkinson disease and multiple system atrophy. In this perspective, an impaired HRV not only is a risk factor for sudden cardiac death mediated by arrhythmias, but also a potential biomarker for monitoring a progressive decline of the autonomous nervous system...
February 2017: Medical Hypotheses
https://www.readbyqxmd.com/read/28062188/a-comprehensive-analysis-and-immunobiology-of-autoimmune-neurological-syndromes-during-the-zika-virus-outbreak-in-c%C3%A3%C2%BAcuta-colombia
#15
Juan-Manuel Anaya, Yhojan Rodríguez, Diana M Monsalve, Daniel Vega, Ernesto Ojeda, Diana González-Bravo, Mónica Rodríguez-Jiménez, Carlos A Pinto-Díaz, Pablo Chaparro, María L Gunturiz, Aftab A Ansari, M Eric Gershwin, Nicolás Molano-González, Carolina Ramírez-Santana, Yeny Acosta-Ampudia
We have focused on the epidemiology and immunobiology of Zika virus (ZIKV) infection and factors associated with the development of Guillain-Barré syndrome (GBS) and other neurological syndromes in Cúcuta, the capital of North Santander department, Colombia. Data of patients with ZIKV disease reported to the national population-based surveillance system were used to calculate the basic reproduction number (R0) and the attack rates (ARs) as well as to develop epidemiological maps. Patients with neurological syndromes were contacted and their diagnoses were confirmed...
February 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/28050780/clinical-characteristics-comorbidities-and-hospital-outcomes-in-hospitalizations-with-cyclic-vomiting-syndrome-a-nationwide-analysis
#16
Sanjay Bhandari, Thangam Venkatesan
BACKGROUND: Data on cyclic vomiting syndrome (CVS) are limited to studies from tertiary care centers. There is a paucity of information about CVS on a national scale. AIM: To study the clinical characteristics, comorbidities, and hospital outcomes in patients hospitalized with CVS using a nationwide database. METHODS: We identified all hospitalizations associated with a primary diagnosis of CVS in 2010 and 2011 using the Nationwide Inpatient Sample with an age category of 18-55 years...
January 3, 2017: Digestive Diseases and Sciences
https://www.readbyqxmd.com/read/28032408/onset-and-evolution-of-anxiety-in-parkinson-s-disease
#17
K Zhu, J J van Hilten, J Marinus
BACKGROUND AND PURPOSE: Anxiety is common in Parkinson's disease (PD) and has a great influence on quality of life. However, little is known about risk factors for development of anxiety in PD. We investigated which factors were associated with longitudinal changes in severity of anxiety symptoms and development of future anxiety in patients who were not anxious at baseline. METHODS: Analyses were performed on data of the PROfiling PARKinson's disease (PROPARK) cohort, a 5-year hospital-based longitudinal cohort of over 400 patients with PD who have been examined annually...
February 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28027135/caring-for-adolescents-with-familial-dysautonomia
#18
Richard D Keegan
No abstract text is available yet for this article.
January 2017: Nursing
https://www.readbyqxmd.com/read/28013357/physical-activity-but-not-sedentary-activity-is-reduced-in-primary-sj%C3%A3-gren-s-syndrome
#19
Wan-Fai Ng, Ariana Miller, Simon J Bowman, Elizabeth J Price, George D Kitas, Colin Pease, Paul Emery, Peter Lanyon, John Hunter, Monica Gupta, Ian Giles, David Isenberg, John McLaren, Marian Regan, Annie Cooper, Steven A Young-Min, Neil McHugh, Saravanan Vadivelu, Robert J Moots, David Coady, Kirsten MacKay, Bhaskar Dasgupta, Nurhan Sutcliffe, Michele Bombardieri, Costantino Pitzalis, Bridget Griffiths, Sheryl Mitchell, Samira Tatiyama Miyamoto, Michael Trenell
The aim of the study was to evaluate the levels of physical activity in individuals with primary Sjögren's syndrome (PSS) and its relationship to the clinical features of PSS. To this cross-sectional study, self-reported levels of physical activity from 273 PSS patients were measured using the International Physical Activity Questionnaire-short form (IPAQ-SF) and were compared with healthy controls matched for age, sex and body mass index. Fatigue and other clinical aspects of PSS including disease status, dryness, daytime sleepiness, dysautonomia, anxiety and depression were assessed using validated tools...
December 24, 2016: Rheumatology International
https://www.readbyqxmd.com/read/28009770/fatal-morvan-syndrome-associated-with-myasthenia-gravis
#20
Madhu Nagappa, Anita Mahadevan, Sanjib Sinha, Parayil S Bindu, Pavagada S Mathuranath, Cheminikara Bineesh, Rose D Bharath, Arun B Taly
INTRODUCTION: Morvan syndrome is a rare and complex autoimmune disorder affecting multiple sites of neuraxis. CASE REPORT: We present fulminant Morvan syndrome, developing on a background of chronic myasthenia gravis. A 54-year-old gentleman presented with fluctuating ophthalmoplegia and proximal muscles weakness of 7 years duration that remitted with pyridostigmine and prednisolone. He developed insomnia of 2 months duration, worsening of myasthenic symptoms and respiratory distress, dysautonomia, encephalopathy, and peripheral nerve hyperexcitability...
January 2017: Neurologist
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