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Autonomic Neuropathy

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https://www.readbyqxmd.com/read/27913968/post-ganglionic-autonomic-neuropathy-associated-with-anti-glutamic-acid-decarboxylase-antibodies
#1
E Fileccia, R Rinaldi, R Liguori, A Incensi, R D'Angelo, M P Giannoccaro, V Donadio
PURPOSE: Antibodies to glutamic acid decarboxylase (GAD-Abs) have been associated with several conditions, rarely involving the autonomic nervous system. Here, we describe two patients complaining of autonomic symptoms in whom a post-ganglionic autonomic neuropathy has been demonstrated in association with significantly elevated serum and CSF GAD-Abs levels. METHODS: Patients underwent nerve conduction studies, sympathetic skin response testing, evaluation of autonomic control of the cardiovascular system and skin biopsy...
December 2, 2016: Clinical Autonomic Research: Official Journal of the Clinical Autonomic Research Society
https://www.readbyqxmd.com/read/27904477/axon-injury-induced-endoplasmic-reticulum-stress-and-neurodegeneration
#2
REVIEW
Yang Hu
Injury to central nervous system axons is a common early characteristic of neurodegenerative diseases. Depending on its location and the type of neuron, axon injury often leads to axon degeneration, retrograde neuronal cell death and progressive permanent loss of vital neuronal functions. Although these sequential events are clearly connected, ample evidence indicates that neuronal soma and axon degenerations are active autonomous processes with distinct molecular mechanisms. By exploiting the anatomical and technical advantages of the retinal ganglion cell (RGC)/optic nerve (ON) system, we demonstrated that inhibition of the PERK-eIF2α-CHOP pathway and activation of the X-box binding protein 1 pathway synergistically protect RGC soma and axon, and preserve visual function, in both acute ON traumatic injury and chronic glaucomatous neuropathy...
October 2016: Neural Regeneration Research
https://www.readbyqxmd.com/read/27898216/characterization-of-prostacyclin-associated-leg-pain-in-patients-with-pulmonary-arterial-hypertension
#3
Gabriel L Pagani-Estévez, Keith M Swetz, Michael D McGoon, Robert P Frantz, Susan K Tointon, Ann M Karnyski, Louise A Durst, James C Watson
RATIONALE: Prostacyclin-associated leg pain is a potentially debilitating adverse effect of prostacyclin therapy for patients with pulmonary arterial hypertension (PAH). However, to our knowledge, this entity has not been systematically studied. OBJECTIVE: To characterize the clinical features and metabolic risk factors for prostacyclinassociated leg pain. METHODS: At one academic medical center we assembled and analyzed a case series of patients with PAH and prostacyclin-associated leg pain...
November 29, 2016: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/27894750/risk-factors-for-autonomic-and-somatic-nerve-dysfunction-in-different-stages-of-glucose-tolerance
#4
Rumyana Dimova, Tsvetalina Tankova, Velina Guergueltcheva, Ivailo Tournev, Nevena Chakarova, Greta Grozeva, Lilia Dakovska
AIM: The present study evaluates autonomic and somatic nerve function in different stages of glucose tolerance and its correlation with different cardio-metabolic parameters. MATERIAL AND METHODS: Four hundred seventy-eight subjects, mean age 49.3±13.7years and mean BMI 31.0±6.2kg/m2, divided according to glucose tolerance: 130 with normal glucose tolerance (NGT), 227 with prediabetes (125 with impaired fasting glucose (IFG) and 102 with isolated impaired glucose tolerance (iIGT)), and 121 with newly-diagnosed T2D (NDT2D), were enrolled...
November 6, 2016: Journal of Diabetes and its Complications
https://www.readbyqxmd.com/read/27894444/unusual-structural-autonomic-disorders-presenting-in-pediatrics-disorders-associated-with-hypoventilation-and-autonomic-neuropathies
#5
REVIEW
Gisela Chelimsky, Thomas Chelimsky
Structural autonomic disorders (producing structural damage to the autonomic nervous system or autonomic centers) are far less common than functional autonomic disorders (reflected in abnormal function of a fundamentally normal autonomic nervous system) in children and teenagers. This article focuses on this uncommon first group in the pediatric clinic. These disorders are grouped into 2 main categories: those characterized by hypoventilation and those that feature an autonomic neuropathy.
February 2017: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/27881717/localization-of-1-deoxysphingolipids-to-mitochondria-induces-mitochondrial-dysfunction
#6
Irina Alecu, Andrea Tedeschi, Natascha Behler, Klaus Wunderling, Christian Lamberz, Mario A R Lauterbach, Anne Gaebler, Daniela Ernst, Paul P Van Veldhoven, Ashraf Al-Amoudi, Eicke Latz, Alaa Othman, Lars Kuerschner, Thorsten Hornemann, Frank Bradke, Christoph Thiele, Anke Penno
1-Deoxysphingolipids (deoxySLs) are atypical sphingolipids that are elevated in the plasma of patients with type 2 diabetes and hereditary sensory and autonomic neuropathy type 1 (HSAN1). Clinically, diabetic neuropathy and HSAN1 are very similar, suggesting the involvement of deoxySLs in the pathology of both diseases. However, very little is known about the biology of these lipids and the underlying pathomechanism. We synthesized an alkyne analogue of 1-deoxysphinganine (doxSA), the metabolic precursor of all deoxySLs, to trace the metabolism and localization of deoxySLs...
November 23, 2016: Journal of Lipid Research
https://www.readbyqxmd.com/read/27869457/autosomal-dominant-cerebellar-ataxia-deafness-and-narcolepsy-adca-dn-associated-with-progressive-cognitive-and-behavioral-deterioration
#7
Lisa A S Walker, Pierre Bourque, Andra M Smith, Jodi Warman Chardon
Objective: Autosomal dominant cerebellar ataxia with deafness and narcolepsy (ADCA-DN) is an emerging syndrome caused by mutations in the C-terminus end of the TS domain of the DNMT1 gene. ADCA-DN is also associated with sensorimotor polyneuropathy, extrapyramidal, and dysautonomic signs, as well as dementia. Little has been reported about the progressive cognitive impairment associated with ADCA-DN. Our objective is to provide a detailed characterization of the cognitive profile of ADCA-DN. Method: Three members of a kindred with ADCA-DN underwent comprehensive neuropsychological testing and neuroimaging...
November 21, 2016: Neuropsychology
https://www.readbyqxmd.com/read/27867500/neurological-complications-in-chronic-kidney-disease
#8
REVIEW
Ria Arnold, Tushar Issar, Arun V Krishnan, Bruce A Pussell
Patients with chronic kidney disease (CKD) are frequently afflicted with neurological complications. These complications can potentially affect both the central and peripheral nervous systems. Common neurological complications in CKD include stroke, cognitive dysfunction, encephalopathy, peripheral and autonomic neuropathies. These conditions have significant impact not only on patient morbidity but also on mortality risk through a variety of mechanisms. Understanding the pathophysiological mechanisms of these conditions can provide insights into effective management strategies for neurological complications...
January 2016: JRSM Cardiovascular Disease
https://www.readbyqxmd.com/read/27843562/risk-factors-of-diabetic-cardiac-autonomic-neuropathy-in-patients-with-type-1-diabetes-mellitus-a-meta-analysis
#9
Mohamed D Dafaalla, Mohammed N Nimir, Mosab I Mohammed, Omer A Ali, Abbashar Hussein
OBJECTIVES: We aimed to stratify the possible risk factors for diabetic cardiac autonomic neuropathy (CAN). METHODS: We did a meta-analysis of risk factors of CAN. We did a web-based search for literature in MEDLINE/PubMed, Scopus database and CENTRAL database up to August 2015. We included clinical trials or cohort studies that provide data about relationship between CAN and variables of interest. Our risk factors of interest were age, sex, duration of diabetes, body mass index (BMI), systolic blood pressure (sBP) and diastolic blood pressure (dBP), glycated haemoglobin (HbA1c), high-density lipoprotein and low-density lipoprotein (HDL and LDL), triglycerides, retinopathy and nephropathy...
2016: Open Heart
https://www.readbyqxmd.com/read/27818945/retrograde-ejaculation-a-rare-presenting-symptom-of-type-1-diabetes-mellitus
#10
Jonathan Kam, Venessa H Tsang, Venu Chalasani
A 19 year old presented with a progressive decline in ejaculate volume over 2 weeks, followed by a complete absence of ejaculate emission. A post-ejaculatory urine specimen demonstrated spermatozoa confirming a diagnosis of retrograde ejaculation. Investigations revealed a raised blood glucose level of 24.5 mmol/L and HbA1c >15%, with positive tests for anti-GAD antibodies and anti-IA2 antibodies consistent with a diagnosis of Type 1 diabetes mellitus. Retrograde ejaculation in diabetes is associated with autonomic neuropathy and is a late feature of the disease...
January 2017: Urology Case Reports
https://www.readbyqxmd.com/read/27814471/reference-data-of-the-european-organisation-for-research-and-treatment-of-cancer-eortc-qlq-cipn20-questionnaire-in-the-general-dutch-population
#11
Floortje Mols, Lonneke V van de Poll-Franse, Gerard Vreugdenhil, Antoinetta J Beijers, Jacobien M Kieffer, Neil K Aaronson, Olga Husson
OBJECTIVE: Chemotherapy-induced peripheral neuropathy (CIPN) is a debilitating side-effect of chemotherapy. However, CIPN symptoms are also reported by patients not receiving chemotherapy. Normative data could help interpret CIPN among cancer patients. Our aim was to generate normative data for the European Organisation for Research and Treatment of Cancer (EORTC) QLQ-CIPN20 Questionnaire designed to assess CIPN from the patients' perspective. The normative CIPN data have also been generated for stratified subgroups formed on the basis of sex, age and comorbidity...
November 1, 2016: European Journal of Cancer
https://www.readbyqxmd.com/read/27803120/cardiovascular-autonomic-neuropathy-and-cardiovascular-outcomes-in-the-diabetes-control-and-complications-trial-epidemiology-of-diabetes-interventions-and-complications-dcct-edic-study
#12
Rodica Pop-Busui, Barbara H Braffett, Bernie Zinman, Catherine Martin, Neil H White, William H Herman, Saul Genuth, Rose Gubitosi-Klug
OBJECTIVE: To examine whether cardiovascular autonomic neuropathy (CAN) is an independent risk factor of cardiovascular disease (CVD) events during DCCT/EDIC. RESEARCH DESIGN AND METHODS: Standardized cardiovascular autonomic reflex tests (R-R response to paced breathing, Valsalva maneuver, postural changes in blood pressure) were performed at DCCT baseline, every 2 years throughout DCCT, and at two time points in EDIC. CVD events were ascertained throughout the study and adjudicated by a review committee...
November 1, 2016: Diabetes Care
https://www.readbyqxmd.com/read/27793437/familial-amyloidosis-with-polyneuropathy-type-1-caused-by-transthyretin-mutation-val50met-val30met-4-cases-in-a-non-endemic-area
#13
N Andrés, J J Poza, J F Martí Massó
INTRODUCTION: Transthyretin-related familial amyloid polyneuropathy (TTR-FAP) typically arises as an autonomic neuropathy primarily affecting small fibres and it occurs in adult patients in their second or third decades of life. It progresses rapidly and can lead to death in approximately 10 years. Other phenotypes have been described in non-endemic areas. OBJECTIVES AND METHODS: We described 4 cases from the Spanish province of Guipuzcoa, a non-endemic area, to highlight the clinical variability of this disease...
October 26, 2016: Neurología: Publicación Oficial de la Sociedad Española de Neurología
https://www.readbyqxmd.com/read/27788152/impact-of-diabetes-type-1-in-children-on-autonomic-modulation-at-rest-and-in-response-to-the-active-orthostatic-test
#14
Thais Roque Giacon, Franciele Marques Vanderlei, Diego Giulliano Destro Christofaro, Luiz Carlos Marques Vanderlei
INTRODUCTION: Cardiovascular autonomic neuropathy is one of the most common complications of diabetes mellitus type 1 (DM1), of which one of the first subclinical manifestations is changes in heart rate variability (HRV). Thus, analysis of HRV associated with the autonomic active orthostatic test is important in this population. OBJECTIVES: To analyze the autonomic modulation responses induced by the implementation of the active orthostatic test, in children with DM1, and study the autonomic modulation by means of HRV indices...
2016: PloS One
https://www.readbyqxmd.com/read/27783183/association-of-sudomotor-function-with-peripheral-artery-disease-in-type-2-diabetes
#15
Simran Chahal, Kanchan Vohra, Ashit Syngle
Peripheral artery disease (PAD) is the major risk factor for cardiovascular disease and lower extremity amputation in patients with diabetes. Autonomic neuropathy is a risk factor for cardiovascular-related morbidity and mortality. Sudomotor dysfunction is well established in type 2 diabetes mellitus (T2DM) and reflects small fibre neuropathy, cardiovascular autonomic neuropathy and peripheral sympathetic autonomic neuropathy. However, the relationship between sudomotor dysfunction and PAD remains unexplored...
October 25, 2016: Neurological Sciences
https://www.readbyqxmd.com/read/27781938/editorial-limited-utility-of-the-handgrip-test-for-the-diagnosis-of-diabetic-cardiovascular-autonomic-neuropathy-there-s-time-enough-but-none-to-spare
#16
K Pafili, E Maltezos, N Papanas
No abstract text is available yet for this article.
October 19, 2016: Current Vascular Pharmacology
https://www.readbyqxmd.com/read/27776522/inositol-1-4-5-trisphosphate-receptor-type-1-autoantibodies-in-paraneoplastic-and-non-paraneoplastic-peripheral-neuropathy
#17
Sven Jarius, Marius Ringelstein, Jürgen Haas, Irina I Serysheva, Lars Komorowski, Kai Fechner, Klaus-Peter Wandinger, Philipp Albrecht, Harald Hefter, Andreas Moser, Eva Neuen-Jacob, Hans-Peter Hartung, Brigitte Wildemann, Orhan Aktas
BACKGROUND: Recently, we described a novel autoantibody, anti-Sj/ITPR1-IgG, that targets the inositol 1,4,5-trisphosphate receptor type 1 (ITPR1) in patients with cerebellar ataxia. However, ITPR1 is expressed not only by Purkinje cells but also in the anterior horn of the spinal cord, in the substantia gelatinosa and in the motor, sensory (including the dorsal root ganglia) and autonomic peripheral nervous system, suggesting that the clinical spectrum associated with autoimmunity to ITPR1 may be broader than initially thought...
October 24, 2016: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/27772781/congenital-insensitivity-to-pain-with-anhidrosis-a-report-of-two-siblings-with-a-novel-mutation-in-trka-ntrk1-gene-in-a-saudi-family
#18
Hussein Algahtani, Muhammad Imran Naseer, Mohammad Al-Qahtani, Shireen Abubakr Abdulrahman, Faisal Boker, Bader Shirah
Congenital insensitivity to pain with anhidrosis (CIPA) or hereditary sensory and autonomic neuropathy type IV (HSAN type IV) is an extremely rare autosomal recessive disorder with an estimated incidence of 1 in 25,000. It was first described in 1963, and since then several case reports and review articles have been published. In this article, we report two brothers with clinical features of CIPA, who presented with recurrent episodes of hyperthermia, anhidrosis, profound loss of pain sensitivity, and unconscious self-mutilation of fingers, lip and tongue...
November 15, 2016: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/27772759/congenital-insensitivity-to-pain-and-anhidrosis-case-report-and-review-of-findings-along-neuro-immune-axis-in-the-disorder
#19
Aditi Vian Varma, Lori McBride, Michael Marble, Ann Tilton
Congenital insensitivity to pain and anhidrosis (CIPA) is one of the hereditary autonomic and sensory neuropathies. Typically presenting in infancy, it manifests as hyperpyrexia from defects in sweating (autonomic) and self-mutilating injuries from pain insensitivity (sensory). CIPA being rare in North America, diagnosis is often missed due to variable presentation. Subsequent management of its complications is therefore delayed. We report an unusual presentation in a 2-year-old girl with preexisting diagnosis of CIPA who was evaluated for bilateral upper extremity paresis of insidious onset...
November 15, 2016: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/27766580/urogenital-autonomic-dysfunction-in-diabetes
#20
Barbara H Braffett, Hunter Wessells, Aruna V Sarma
This review details the epidemiology, possible mechanisms, and risk factors associated with urogenital autonomic dysfunction in diabetes. Autonomic neuropathy in diabetes is associated with various urological complications including bladder and sexual dysfunction. Several studies have reported the high prevalence of bladder and sexual dysfunction in both men and women. The DCCT/EDIC UroEDIC study examined the association between cardiovascular autonomic neuropathy and bladder and sexual dysfunction in a large cohort of participants with type 1 diabetes and was the first to report significant associations...
December 2016: Current Diabetes Reports
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