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Ganglionopathies

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https://www.readbyqxmd.com/read/27861227/sensory-ganglionopathy-associated-with-sj%C3%A3-gren-syndrome
#1
Bhavesh Trikamji, Nastaran Rafiei, Hadi Mohammadkhanli, Shri K Mishra
No abstract text is available yet for this article.
December 2016: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/27859440/peripheral-nerve-ultrasound-in-cerebellar-ataxia-neuropathy-vestibular-areflexia-syndrome-canvas
#2
Luciana Pelosi, Ruth Leadbetter, Eoin Mulroy, Andrew M Chancellor, Stuart Mossman, Richard Roxburgh
INTRODUCTION We report preliminary findings of nerve ultrasound in patients with Cerebellar Ataxia Neuropathy Vestibular Areflexia Syndrome (CANVAS) who have sensory impairment due to dorsal root ganglionopathy. METHODS The ultrasound cross sectional area (CSA) of median and ulnar nerves of 7 CANVAS patients was compared with 7 age- and gender-matched controls and with the mean CSA of our reference population. RESULTS The nerve CSA of CANVAS patients was significantly smaller than controls at all sites (P < 0...
November 15, 2016: Muscle & Nerve
https://www.readbyqxmd.com/read/27704495/neuromuscular-diseases
#3
Brett M Morrison
Neuromuscular diseases are a broadly defined group of disorders that all involve injury or dysfunction of peripheral nerves or muscle. The site of injury can be in the cell bodies (i.e., amyotrophic lateral sclerosis [ALS] or sensory ganglionopathies), axons (i.e., axonal peripheral neuropathies or brachial plexopathies), Schwann cells (i.e., chronic inflammatory demyelinating polyradiculoneuropathy), neuromuscular junction (i.e., myasthenia gravis or Lambert-Eaton myasthenic syndrome), muscle (i.e., inflammatory myopathy or muscular dystrophy), or any combination of these sites...
October 2016: Seminars in Neurology
https://www.readbyqxmd.com/read/27301520/brachial-diparesis-due-to-motor-neuronopathy-as-one-of-the-predominant-presenting-signs-of-occult-small-cell-lung-carcinoma
#4
Sefik Evren Erdener, Atay Vural, Cagri Mesut Temucin, Sevim Erdem Ozdamar, Gulay Nurlu, Kubilay Varli, Nese Dericioglu
Sensory neuronopathy is a well-established presentation in paraneoplastic neurological syndromes that is mostly associated with small cell lung cancer and anti-Hu antibodies. Motor neuronopathy, on the other hand, is an extremely rare observation in this syndrome. A 56-year-old man presented with asymmetric brachial diparesis and sensory ataxia. Electrophysiological studies revealed sensory ganglionopathy and progressive anterior horn degeneration in cervical segments. Small cell lung carcinoma with associated anti-Hu antibodies was later diagnosed...
2016: Internal Medicine
https://www.readbyqxmd.com/read/27175675/sj%C3%A3-gren-sensory-neuronopathy-sj%C3%A3-gren-ganglionopathy-long-term-outcome-and-treatment-response-in-a-series-of-13-cases
#5
P Ricardo Pereira, Karine Viala, Thierry Maisonobe, Julien Haroche, Alexis Mathian, Miguel Hié, Zahir Amoura, Fleur Cohen Aubart
Primary Sjögren syndrome (SS) is an autoimmune disease mainly affecting the exocrine glands causing a sicca syndrome. Neurological manifestations are rarely seen in SS although they are debilitating. Peripheral neuropathies namely sensory axonal neuropathy and painful small fiber neuropathy are the most frequent neurological manifestations. Sensory neuronopathy (SN) is less frequently seen although leading to more severe handicap.The aim of the study was to analyze the clinical presentation and treatment efficacy in a series of SS-related SN...
May 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27112689/autoimmune-autonomic-disorders
#6
Andrew Mckeon, Eduardo E Benarroch
Autoimmune autonomic disorders occur because of an immune response directed against sympathetic, parasympathetic, and enteric ganglia, autonomic nerves, or central autonomic pathways. In general, peripheral autoimmune disorders manifest with either generalized or restricted autonomic failure, whereas central autoimmune disorders manifest primarily with autonomic hyperactivity. Some autonomic disorders are generalized, and others are limited in their anatomic extent, e.g., isolated gastrointestinal dysmotility...
2016: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/26807576/association-between-anti-ganglionic-nicotinic-acetylcholine-receptor-gachr-antibodies-and-hla-drb1-alleles-in-the-japanese-population
#7
Yasuhiro Maeda, Kiyoshi Migita, Osamu Higuchi, Akihiro Mukaino, Hiroshi Furukawa, Atsumasa Komori, Minoru Nakamura, Satoru Hashimoto, Shinya Nagaoka, Seigo Abiru, Hiroshi Yatsuhashi, Hidenori Matsuo, Atsushi Kawakami, Michio Yasunami, Shunya Nakane
BACKGROUND/AIMS: Anti-ganglionic nicotinic acetylcholine receptor (gAChR) antibodies are observed in autoimmune diseases, as well as in patients with autoimmune autonomic ganglionopathy. However, the genetic background of anti-gAChR antibodies is unclear. Here, we investigated HLA alleles in autoimmune hepatitis (AIH) patients with or without anti-gAChR antibodies. METHODOLOGY/PRINCIPAL FINDINGS: Genomic DNA from 260 patients with type-1 autoimmune hepatitis (AIH) were genotyped for HLA-A, B, DRB1, and DQB1 loci...
2016: PloS One
https://www.readbyqxmd.com/read/26802964/a-novel-use-of-n-acetylcystine-for-the-management-of-recurrent-small-bowel-pseudo-obstruction-in-a-patient-with-autoimmune-autonomic-ganglionopathy
#8
Shahryar Tork, Scott R Kelley, Kevin M Tymitz
No abstract text is available yet for this article.
May 2015: American Surgeon
https://www.readbyqxmd.com/read/26795713/sensory-ganglionopathy-and-the-blink-reflex-electrophysiological-features
#9
Taimour Alam, Alexander StJ E Barker, James J P Alix, Marios Hadjivassiliou, Dasappaiah G Rao
BACKGROUND: Sensory ganglionopathy (SG) is characterised by asymmetrical sensory fibre degeneration, with the primary pathology occurring at the level of the dorsal root ganglion. It is seen in the context of autoimmune, paraneoplastic, and degenerative disorders. There is limited literature examining the electrophysiological correlate of the trigeminal ganglion and associated pathways, the blink reflex (BR), in cases of SG. Previous work has suggested that the BR is preserved in cases of SG associated with paraneoplasia...
May 2016: Canadian Journal of Neurological Sciences. le Journal Canadien des Sciences Neurologiques
https://www.readbyqxmd.com/read/26794588/thermoregulatory-disorders-and-illness-related-to-heat-and-cold-stress
#10
REVIEW
William P Cheshire
Thermoregulation is a vital function of the autonomic nervous system in response to cold and heat stress. Thermoregulatory physiology sustains health by keeping body core temperature within a degree or two of 37°C, which enables normal cellular function. Heat production and dissipation are dependent on a coordinated set of autonomic responses. The clinical detection of thermoregulatory impairment provides important diagnostic and localizing information in the evaluation of disorders that impair thermoregulatory pathways, including autonomic neuropathies and ganglionopathies...
April 2016: Autonomic Neuroscience: Basic & Clinical
https://www.readbyqxmd.com/read/26698638/autonomic-and-sensory-ganglionopathy-occurring-in-a-patient-with-fulminant-type-1-diabetes-mellitus
#11
Mutsuki Makino, Dai Hiwatashi, Kesami Minemura, Kenji Kawaguchi
A 63-year-old male with a previous infection was admitted to our hospital because of acute pancreatitis. Although he had no history of diabetes mellitus, laboratory examinations revealed marked hyperglycemia on admission, and intensive insulin treatment was required. After 2 weeks, he developed severe pandysautonomia and sensory impairment, and eventually died from colonic perforation caused by paralytic ileus at 1 year after onset. Autopsy findings showed a complete loss of pancreatic islet beta cells with mild fibrosis of the exocrine pancreas...
February 2016: Pathology International
https://www.readbyqxmd.com/read/26560953/-acute-sensory-neuropathies-and-acute-autonomic-neuropathies
#12
REVIEW
Haruki Koike
From the perspective of neuropathies with an acute onset mimicking that of Guillain-Barr? syndrome (GBS), cases with profound sensory and/or autonomic impairment without any significant weakness have been reported. Although the possibility of infectious or toxic etiologies should be carefully excluded, immune mechanisms similar to those in GBS are suggested to be involved in these so-called acute sensory neuropathies and acute autonomic neuropathies. The types of neuropathy include those with predominant sensory manifestations, predominant autonomic manifestations such as autoimmune autonomic ganglionopathy, and both sensory and autonomic manifestations such as acute autonomic and sensory neuropathy...
November 2015: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/26526686/immunotherapy-prospects-for-painful-small-fiber-sensory-neuropathies-and-ganglionopathies
#13
REVIEW
Anne Louise Oaklander
The best-known peripheral neuropathies are those affecting the large, myelinated motor and sensory fibers. These have well-established immunological causes and therapies. Far less is known about the somatic and autonomic "small fibers"; the unmyelinated C-fibers, thinly myelinated A-deltas, and postganglionic sympathetics. The small fibers sense pain and itch, innervate internal organs and tissues, and modulate the inflammatory and immune responses. Symptoms of small-fiber neuropathy include chronic pain and itch, sensory impairment, edema, and skin color, temperature, and sweating changes...
January 2016: Neurotherapeutics: the Journal of the American Society for Experimental NeuroTherapeutics
https://www.readbyqxmd.com/read/26480742/-autoimmune-autonomic-ganglionopathy-aag
#14
Michiaki Koga
No abstract text is available yet for this article.
September 2015: Nihon Rinsho. Japanese Journal of Clinical Medicine
https://www.readbyqxmd.com/read/26467754/sensory-neuronopathies
#15
REVIEW
Kelly Graham Gwathmey
The sensory neuronopathies (or ganglionopathies) are a small subcategory of neuropathies characterized by primary degeneration of the dorsal root ganglia and trigeminal ganglion sensory neurons, resulting in a distinctive clinical presentation. Patients typically have subacute onset of asymmetric, non-length-dependent sensory impairment and early ataxia. The etiologies of acquired sensory neuronopathies are rather limited. Early identification is imperative, as they may herald an underlying malignancy or an autoimmune condition such as Sjögren syndrome...
January 2016: Muscle & Nerve
https://www.readbyqxmd.com/read/26388117/delayed-onset-friedreich-s-ataxia-revisited
#16
MULTICENTER STUDY
Claire Lecocq, Perrine Charles, Jean-Philippe Azulay, Wassilios Meissner, Myriam Rai, Karine N'Guyen, Yann Péréon, Nelly Fabre, Elsa Robin, Sylvie Courtois, Lucie Guyant-Maréchal, Fabien Zagnoli, Gabrielle Rudolf, Mathilde Renaud, Mathieu Sévin-Allouet, Fabien Lesne, Nick Alaerts, Cyril Goizet, Patrick Calvas, Alexandre Eusebio, Claire Guissart, Pascal Derkinderen, Francois Tison, Alexis Brice, Michel Koenig, Massimo Pandolfo, Christine Tranchant, Alexandra Dürr, Mathieu Anheim
BACKGROUND: Friedreich's ataxia usually occurs before the age of 25. Rare variants have been described, such as late-onset Friedreich's ataxia and very-late-onset Friedreich's ataxia, occurring after 25 and 40 years, respectively. We describe the clinical, functional, and molecular findings from a large series of late-onset Friedreich's ataxia and very-late-onset Friedreich's ataxia and compare them with typical-onset Friedreich's ataxia. METHODS: Phenotypic and genotypic comparison of 44 late-onset Friedreich's ataxia, 30 very late-onset Friedreich's ataxia, and 180 typical Friedreich's ataxia was undertaken...
January 2016: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/26245264/rituximab-based-therapy-and-long-term-control-of-autoimmune-autonomic-ganglionopathy
#17
Arjun Gupta, Samar Harris, Steven Vernino, Harris V Naina
We present a patient with autoimmune autonomic ganglionopathy (AAG) who had persistently positive ganglionic nicotinic acetylcholine receptor antibody levels despite immunosuppressive therapy. Rituximab-based therapy for an incidental lymphoma was associated with prolonged symptomatic and serological control of AAG.
August 2015: Clinical Autonomic Research: Official Journal of the Clinical Autonomic Research Society
https://www.readbyqxmd.com/read/26090802/neuronal-subtype-and-satellite-cell-tropism-are-determinants-of-varicella-zoster-virus-virulence-in-human-dorsal-root-ganglia-xenografts-in-vivo
#18
Leigh Zerboni, Ann Arvin
Varicella zoster virus (VZV), a human alphaherpesvirus, causes varicella during primary infection. VZV reactivation from neuronal latency may cause herpes zoster, post herpetic neuralgia (PHN) and other neurologic syndromes. To investigate VZV neuropathogenesis, we developed a model using human dorsal root ganglia (DRG) xenografts in immunodeficient (SCID) mice. The SCID DRG model provides an opportunity to examine characteristics of VZV infection that occur in the context of the specialized architecture of DRG, in which nerve cell bodies are ensheathed by satellite glial cells (SGC) which support neuronal homeostasis...
June 2015: PLoS Pathogens
https://www.readbyqxmd.com/read/25975318/a-novel-use-of-n-acetylcystine-for-the-management-of-recurrent-small-bowel-pseudo-obstruction-in-a-patient-with-autoimmune-autonomic-ganglionopathy
#19
Shahryar Tork, Scott R Kelley, Kevin M Tymitz
No abstract text is available yet for this article.
May 2015: American Surgeon
https://www.readbyqxmd.com/read/25790156/clinical-features-of-autoimmune-autonomic-ganglionopathy-and-the-detection-of-subunit-specific-autoantibodies-to-the-ganglionic-acetylcholine-receptor-in-japanese-patients
#20
Shunya Nakane, Osamu Higuchi, Michiaki Koga, Takashi Kanda, Kenya Murata, Takashi Suzuki, Hiroko Kurono, Masanari Kunimoto, Ken-ichi Kaida, Akihiro Mukaino, Waka Sakai, Yasuhiro Maeda, Hidenori Matsuo
Autoimmune autonomic ganglionopathy (AAG) is a rare acquired channelopathy that is characterized by pandysautonomia, in which autoantibodies to ganglionic nicotinic acetylcholine receptors (gAChR) may play a central role. Radioimmunoprecipitation (RIP) assays have been used for the sensitive detection of autoantibodies to gAChR in the serum of patients with AAG. Here, we developed luciferase immunoprecipitation systems (LIPS) to diagnose AAG based on IgGs to both the α3 and β4 gAChR subunits in patient serum...
2015: PloS One
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