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Dysautonomia

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https://www.readbyqxmd.com/read/28906019/response-letter-to-optimising-physiology-for-adolescents-with-dysautonomia
#1
K Armstrong, A M De Souza, P Sneddon, J E Potts, V E Claydon, S Sanatani
We would like to thank Dr Thaxter Nesbeth (1) for her interest and insightful comments on our paper entitled, "Exercise and the multidisciplinary holistic approach to adolescent dysautonomia"(2). We agree that recurrent orthostatic intolerance is often best managed through lifestyle modification rather than pharmacologic intervention (at least as an initial step), and as such in the present study we evaluated the impact of our exercise programme incorporated in addition to our standard clinical multidisciplinary management approach...
September 14, 2017: Acta Paediatrica
https://www.readbyqxmd.com/read/28904566/is-vitamin-d-deficiency-implicated-in-autonomic-dysfunction
#2
REVIEW
Rozina Wadhwania
Dysautonomia, dysfunction of the autonomic nervous system, presents with heterogeneous clinical features from an imbalanced regulation of the sympathetic and parasympathetic nervous systems. Low Vitamin D levels can explain the heterogeneous clinical features of migraine headaches, cardiac and gastrointestinal dysfunction, and oxidative stress evident in dysautonomia patients. The role of Vitamin D in modulating pain sensitivity has been recently established. However, there is a lack of research and understanding regarding the association between Vitamin D deficiency and autonomic dysfunction...
April 2017: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/28901332/prolonged-hypotension-associated-with-wernicke-s-encephalopathy
#3
H Maeda, A Kuriyama, T Tanaka
A diagnosis of Wernicke's encephalopathy (WE) is difficult. Transient hypotension has been reported as a rare complication of WE. We herein report a case of prolonged hypotension and dysuautonomia associated with WE in a 69-year-old man with underlying alcohol abuse. Without apparent etiology of shock, this patient remained hypotensive for 9 days, requiring a vasopressor, despite daily administration of thiamine 600 mg. Fluctuation of blood pressure caused by postural change and bradycardia in the presence of shock indicated that this patient had dysautonomia...
September 13, 2017: European Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/28879885/anti-ganglioside-antibodies-profile-in-guillain-barr%C3%A3-syndrome-correlation-with-clinical-features-electrophysiological-pattern-and-outcome
#4
G Sivaram Naik, A Kanikannan Meena, B Ashok Kumar Reddy, Rukmini K Mridula, Shaik A Jabeen, Rupam Borgohain
BACKGROUND: Guillain-Barré syndrome (GBS) and its subtypes are associated with distinct anti-ganglioside antibodies. Hence, we aimed to determine the frequency of anti-ganglioside antibodies and its correlation with clinical features, electrophysiological patterns, and outcome in patients with GBS. MATERIAL AND METHODS: The data regarding clinical features, electrophysiological patterns, and outcome at 6 months were collected and analyzed from the case records of patients diagnosed with GBS during 2008-2013 at a tertiary care hospital in south India...
September 2017: Neurology India
https://www.readbyqxmd.com/read/28870420/value-of-protein-concentration-in-cerebrospinal-fluid-in-paediatric-patients-with-guillain-barre-syndrome
#5
Martha Esther Vidrio-Becerra, Jaime Valle-Leal, María Enriqueta Loaiza-Sarabia, Lucia Alvarez-Bastidas, Jesus Ignacio Lachica-Valle, Cruz Mónica López-Morales
INTRODUCTION AND OBJECTIVE: The albumin-cytologic dissociation in cerebrospinal fluid (CSF) supports the diagnosis of Guillain-Barre syndrome (GBS) but does not support the prognosis, so the aim of this study is to determine the usefulness of protein numbers in the CSF to predict progression in paediatric patients. PATIENTS AND METHODS: A diagnostic test was performed in paediatric patients with GBS, analysing sociodemographic, clinical and protein variables in CSF as well as electromyography...
September 1, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/28827489/pyridoxine-toxicity-small-fiber-neuropathy-with-dysautonomia-a-case-report
#6
Rae Bacharach, Max Lowden, Aiesha Ahmed
Pyridoxine (vitamin B6) toxicity is a well-known cause of primary sensory, length-dependent, axonal polyneuropathy. Although sensory symptoms predominate, autonomic symptoms have also been reported in some cases. To date, there is no objective evidence of autonomic dysfunction reported in the literature. We present the case of a 41-year-old woman with 2 years of progressive burning pain, numbness, tingling, and weakness in a stocking-glove distribution who was found to have severe pyridoxine toxicity. Concurrent presence of large and small fiber nerve dysfunction was noted in the form of abnormal electromyography/nerve conduction study demonstrating a chronic sensory polyneuropathy and autonomic testing demonstrating abnormal responses to quantitative sweat testing and cardiovagal function testing...
September 2017: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/28800081/autonomic-dysfunction-after-mild-traumatic-brain-injury
#7
Dmitry Esterov, Brian D Greenwald
A mild traumatic brain injury (mTBI) is a complex pathophysiologic process that has a systemic effect on the body aside from solely an impairment in cognitive function. Dysfunction of the autonomic nervous system (ANS) has been found to induce abnormalities in organ systems throughout the body, and may contribute to cardiovascular dysregulation and increased mortality. Autonomic dysfunction, also known as dysautonomia, has been studied in moderate and severe TBI, and has emerged as a major contributing factor in the symptomatology in mTBI as well...
August 11, 2017: Brain Sciences
https://www.readbyqxmd.com/read/28795253/autonomic-responses-to-head-up-tilt-test-in-children-with-autism-spectrum-disorders
#8
Véronique-Aurélie Bricout, Marion Pace, Léa Dumortier, Anne Favre-Juvin, Michel Guinot
Autism Spectrum Disorders (ASD) is a group of neurodevelopmental disorders often manifested by social and behavioral deficiencies. Autonomic dysfunction is frequently reported in the autistic population but the mechanisms remain largely unknown. We aimed to characterize the cardiac autonomic profile of children with autism during a head-up tilt test. Thirty-nine male children were recruited: 19 controls (9.9 ± 1.6 years) and 20 children with ASD without intellectual disability (10.7 ± 1.2 years). Each child underwent a head-up tilt test on a motorized tilt table...
August 9, 2017: Journal of Abnormal Child Psychology
https://www.readbyqxmd.com/read/28770651/multiple-system-atrophy-many-lessons-from-the-transcriptome
#9
Ashton Curry-Hyde, Bei Jun Chen, Uwe Ueberham, Thomas Arendt, Michael Janitz
Multiple system atrophy (MSA) is a complex, multifactorial, debilitating neurodegenerative disease that is often misdiagnosed and misunderstood. MSA has two subclasses, MSA-P and MSA-C, defined by the dominance of parkinsonism or cerebellar dysfunction in the earlier stages of disease, coupled with dysautonomia. This distinction between subclasses becomes largely redundant as the disease progresses. Aggregation of α-synuclein is a clinical marker used to confirm MSA diagnoses, which can only be performed postmortem...
August 1, 2017: Neuroscientist: a Review Journal Bringing Neurobiology, Neurology and Psychiatry
https://www.readbyqxmd.com/read/28761904/iglon5-antibody-neurological-accompaniments-and-outcomes-in-20-patients
#10
Josephe A Honorat, Lars Komorowski, Keith A Josephs, Kai Fechner, Erik K St Louis, Shannon R Hinson, Sabine Lederer, Neeraj Kumar, Avi Gadoth, Vanda A Lennon, Sean J Pittock, Andrew McKeon
OBJECTIVE: To describe the phenotypes, treatment response, and outcome of IgLON5 autoimmunity. METHODS: Archived serum and CSF specimens from 367 patients known to harbor unclassified antibodies which stained neural synapses diffusely (mimicking amphiphysin-IgG) were reevaluated by indirect immunofluorescence assay (IFA) using a composite of mouse tissues and recombinant IgLON5-transfected cell-based assay (CBA, Euroimmun). RESULTS: Available specimens (serum, 25; CSF, 9) from 26/367 patients (7%) had identical IFA appearance and robust IgLON5 CBA positivity...
September 2017: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/28738696/the-role-of-autoantibodies-in-the-syndromes-of-orthostatic-intolerance-a-systematic-review
#11
REVIEW
Mohammed Ruzieh, Lillian Batizy, Osama Dasa, Carson Oostra, Blair Grubb
Orthostatic intolerance is defined as the provocation of symptoms upon standing, commonly caused by neurogenic orthostatic hypotension (OH) and postural tachycardia syndrome (POTS), the etiology for which has not been fully uncovered yet. Many reports have described the occurrence of dysautonomia, orthostatic intolerance and POTS following febrile illness, presumably viral and post-vaccine. Furthermore, patients with dysautonomia have higher rates of autoimmune disorders such as Hashimoto thyroiditis and SLE...
October 2017: Scandinavian Cardiovascular Journal: SCJ
https://www.readbyqxmd.com/read/28725706/the-effect-of-deep-brain-stimulation-on-the-non-motor-symptoms-of-parkinson-s-disease-a-critical-review-of-the-current-evidence
#12
REVIEW
Mónica M Kurtis, Thadshani Rajah, Luisa F Delgado, Haidar S Dafsari
The benefit of deep brain stimulation (DBS) in controlling the motor symptoms of Parkinson's disease is well established, however, the impact on the non-motor symptoms (NMS) remains to be elucidated, although the growing investigative efforts are promising. This article reviews the reported data and considers the level of evidence available with regard to the effect of DBS on NMS total burden and on the cognitive, neuropsychiatric, sleep, pain, dysautonomic, and weight domains. Multiple case series suggest that DBS improves the burden of NMS by reducing prevalence, intensity, and non-motor fluctuations...
2017: NPJ Parkinson's Disease
https://www.readbyqxmd.com/read/28717942/erratum-to-animal-and-cellular-models-of-familial-dysautonomia
#13
Frances Lefcort, Marc Mergy, Sarah B Ohlen, Yumi Ueki, Lynn George
No abstract text is available yet for this article.
August 2017: Clinical Autonomic Research: Official Journal of the Clinical Autonomic Research Society
https://www.readbyqxmd.com/read/28716515/calming-the-storm-dysautonomia-for-the-pediatrician
#14
Justin M Burton, Olga M Morozova
Dysautonomia is a potentially life-threatening syndrome seen in many different types of brain injuries. It involves paroxysmal sympathetic hyperactivity and typically includes a constellation of symptoms, including: tachycardia, tachypnea, hyperthermia, hypertension, diaphoresis, hypertonia, and/or decerebrate or decorticate posturing. It is a clinical diagnosis of exclusion. A multimodal treatment approach is necessary including environmental modifications along with pharmacotherapy. Early management can help prevent comorbidities including secondary brain injury while also improving patient outcomes...
July 14, 2017: Current Problems in Pediatric and Adolescent Health Care
https://www.readbyqxmd.com/read/28707017/basic-research-and-model-systems-in-familial-dysautonomia-what-do-we-know-and-what-s-next
#15
EDITORIAL
Antonio Heras-Garvin
No abstract text is available yet for this article.
August 2017: Clinical Autonomic Research: Official Journal of the Clinical Autonomic Research Society
https://www.readbyqxmd.com/read/28674865/intranasal-dexmedetomidine-for-adrenergic-crisis-in-familial-dysautonomia
#16
Christy L Spalink, Erin Barnes, Jose-Alberto Palma, Lucy Norcliffe-Kaufmann, Horacio Kaufmann
PURPOSE: To report the use of intranasal dexmedetomidine, an α2-adrenergic agonist for the acute treatment of refractory adrenergic crisis in patients with familial dysautonomia. METHODS: Case series. RESULTS: Three patients with genetically confirmed familial dysautonomia (case 1: 20-year-old male; case 2: 43-year-old male; case 3: 26-year-old female) received intranasal dexmedetomidine 2 mcg/kg, half of the dose in each nostril, for the acute treatment of adrenergic crisis...
August 2017: Clinical Autonomic Research: Official Journal of the Clinical Autonomic Research Society
https://www.readbyqxmd.com/read/28667575/animal-and-cellular-models-of-familial-dysautonomia
#17
REVIEW
Frances Lefcort, Marc Mergy, Sarah B Ohlen, Yumi Ueki, Lynn George
Since Riley and Day first described the clinical phenotype of patients with familial dysautonomia (FD) over 60 years ago, the field has made considerable progress clinically, scientifically, and translationally in treating and understanding the etiology of FD. FD is classified as a hereditary sensory and autonomic neuropathy (HSAN type III) and is both a developmental and a progressive neurodegenerative condition that results from an autosomal recessive mutation in the gene IKBKAP, also known as ELP1. FD primarily impacts the peripheral nervous system but also manifests in central nervous system disruption, especially in the retina and optic nerve...
August 2017: Clinical Autonomic Research: Official Journal of the Clinical Autonomic Research Society
https://www.readbyqxmd.com/read/28660750/urinary-manifestations-in-isaacs-s-syndrome-our-experience-in-8-cases
#18
Silvia N Gonzalez Primomo, Leandro Blas, Alicia C Bertotti, Carlos Ameri
INTRODUCTION: Isaacs's syndrome (IS), is a rare neurological disorder, characterized by sustained muscular activity, fasciculations, cramps, myokymia, excessive sweating, and occasional elevation of creatine phosphokinase (CPK) enzyme. AIM: To report our experience in patients with IS and urinary manifestations, describing clinical findings, test's results, and response to treatment. Methods An observational, retrospective analysis of patients with IS and urinary manifestations treated at German Hospital of Buenos Aires between 2001 and 2011 was done...
June 29, 2017: Neurourology and Urodynamics
https://www.readbyqxmd.com/read/28651678/latent-cognitive-phenotypes-in-de-novo-parkinson-s-disease-a-person-centered-approach
#19
Denise R LaBelle, Ryan R Walsh, Sarah J Banks
OBJECTIVES: Cognitive impairment is an important aspect of Parkinson's disease (PD), but there is considerable heterogeneity in its presentation. This investigation aims to identify and characterize latent cognitive phenotypes in early PD. METHODS: Latent class analysis, a data-driven, person-centered, cluster analysis was performed on cognitive data from the Parkinson's Progressive Markers Initiative baseline visit. This analytic method facilitates identification of naturally occurring endophenotypes...
June 27, 2017: Journal of the International Neuropsychological Society: JINS
https://www.readbyqxmd.com/read/28648835/effect-of-glp-1-receptor-agonist-on-gastrointestinal-tract-motility-and-residue-rates-as-evaluated-by-capsule-endoscopy
#20
Y Nakatani, M Maeda, M Matsumura, R Shimizu, N Banba, Y Aso, T Yasu, H Harasawa
AIM: This study evaluated the effects of a glucagon-like peptide-1 receptor agonist on gastrointestinal (GI) tract motility and residue rates by examining GI transit time and lumen using capsule endoscopy. MATERIAL AND METHODS: GI motility and lumen were assessed by capsule endoscopy before and after liraglutide administration in 14 patients with type 2 diabetes mellitus (T2DM). RESULTS: Gastric transit time in the group with diabetic neuropathy (DN) was 1:12:36±1:04:30h before liraglutide administration and 0:48:40±0:32:52h after administration (nonsignificant difference, P=0...
June 22, 2017: Diabetes & Metabolism
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