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Ganglionopathies

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https://www.readbyqxmd.com/read/29766276/anti-ganglionic-achr-antibodies-in-japanese-patients-with-motility-disorders
#1
Akihiro Mukaino, Hitomi Minami, Hajime Isomoto, Hitomi Hamamoto, Eikichi Ihara, Yasuhiro Maeda, Osamu Higuchi, Tohru Okanishi, Yohei Kokudo, Kazushi Deguchi, Fumisato Sasaki, Toshihito Ueki, Ken-Ya Murata, Takeshi Yoshida, Mistuyo Kinjo, Yoshihiro Ogawa, Akio Ido, Hidenori Matsuo, Kazuhiko Nakao, Shunya Nakane
BACKGROUND: The existence of several autoantibodies suggests an autoimmune basis for gastrointestinal (GI) dysmotility. Whether GI motility disorders are features of autoimmune autonomic ganglionopathy (AAG) or are related to circulating anti-ganglionic acetylcholine receptor (gAChR) antibodies (Abs) is not known. The aim of this study was to determine the associations between autonomic dysfunction, anti-gAChR Abs, and clinical features in patients with GI motility disorders including achalasia and chronic intestinal pseudo-obstruction (CIPO)...
May 15, 2018: Journal of Gastroenterology
https://www.readbyqxmd.com/read/29685292/seronegative-and-seropositive-autoimmune-autonomic-ganglionopathy-aag-same-clinical-picture-same-response-to-immunotherapy
#2
Beatriz Tijero, Rocio Del Pino, Tomás Pérez-Concha, Maria Angeles Acera, Iñigo Gabilondo, Koldo Berganzo, Frances Graus, Jesus Daniel Martinez-Alday, Joseba Barcena, Juan Carlos Gómez-Esteban
Two patients with a syndrome of pandisautonomia with clinical criteria of AAG are provided. Both patients present a similar clinical picture and response to immunosuppressive treatment. One of them has positive antibodies against the ganglionic nicotinic acetylcholine (gAChr) and the other does not. This brief article serves to reflect the spectrum of AAG, at a clinical level, in laboratory tests and in the response to immunotherapy, independently of the presence of positive gAChr antibodies.
June 15, 2018: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/29661595/excitability-of-sensory-axons-in-amyotrophic-lateral-sclerosis
#3
José Manuel Matamala, James Howells, Thanuja Dharmadasa, William Huynh, Susanna B Park, David Burke, Matthew C Kiernan
OBJECTIVE: To evaluate the excitability of sensory axons in patients with amyotrophic lateral sclerosis (ALS). METHODS: Comprehensive sensory nerve excitability studies were prospectively performed on 28 sporadic ALS patients, compared to age-matched controls. Sensory nerve action potentials were recorded from digit 2 following median nerve stimulation at the wrist. Disease severity was measured using motor unit number estimation (MUNE), the revised ALS Functional Rating Scale (ALSFRS-R) and the MRC scale...
April 2, 2018: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/29578430/development-of-ganglionopathy-and-tabetic-visceral-crises-on-the-background-of-polyradiculoneuropathy-associated-with-monoclonal-gammopathy-case-report
#4
R Svistilnik, N Kostiukova
The article presents an analysis of the clinical occurrence of development of chronic polyradiculoneuropathy associated with monoclonal IgG/k (kappa) gammopathy of the undetermined significance. The peculiarity of this occurrence is the uniqueness of the development of the symptoms which are characteristic of tabes dorsalis in this pathology with episodic severe visceral crises and also with ganglionopathy. The example describes the clinical polymorphism of the course of visceral crises, the problems of their diagnosis and as a consequence of inadequate treatment with the development of severe social maladaptation...
February 2018: Georgian Medical News
https://www.readbyqxmd.com/read/29493845/the-role-of-skin-biopsy-in-differentiating-small-fiber-neuropathy-from-ganglionopathy
#5
V Provitera, C H Gibbons, G Wendelschafer-Crabb, V Donadio, D F Vitale, A Loavenbruck, A Stancanelli, G Caporaso, R Liguori, N Wang, L Santoro, W R Kennedy, M Nolano
BACKGROUND AND PURPOSE: We aimed to test the clinical utility of the leg:thigh intraepidermal nerve-fiber (IENF) density ratio as a parameter to discriminate between length-dependent small-fiber neuropathy (SFN) and small-fiber sensory ganglionopathy (SFSG) in subjects with signs and symptoms of small-fiber pathology. METHODS: We retrospectively evaluated thigh and leg IENF density in 314 subjects with small-fiber pathology (173 with distal symmetrical length-dependent SFN and 141 with non-length-dependent SFSG)...
June 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/29305816/autoimmune-autonomic-neuropathies-time-to-look-beyond-autoimmune-autonomic-ganglionopathy
#6
EDITORIAL
Srikanth Muppidi
No abstract text is available yet for this article.
February 2018: Clinical Autonomic Research: Official Journal of the Clinical Autonomic Research Society
https://www.readbyqxmd.com/read/29299392/rapid-neurophysiological-screening-for-sensory-ganglionopathy-a-novel-approach
#7
Panagiotis Zis, Marios Hadjivassiliou, Ptolemaios Georgios Sarrigiannis, Alexander St John Edward Barker, Dasappaiah Ganesh Rao
Background and Aim: Pure sensory neuropathies involving the dorsal root ganglia are commonly referred to as sensory ganglionopathies (SG). Causes of SG can be inherited (as seen in Friedreich's ataxia) or acquired (e.g. immune-mediated or paraneoplastic). Diagnostic criteria for confirming SG have been published and consist of a combination of clinical and neurophysiological parameters. The aim of our study was to develop a neurophysiological method for rapid screening for diagnosis of SG...
December 2017: Brain and Behavior
https://www.readbyqxmd.com/read/29280036/seronegative-autoimmune-autonomic-neuropathy-a-distinct-clinical-entity
#8
Elisabeth P Golden, Meredith A Bryarly, Steven Vernino
PURPOSE: Autoimmune autonomic ganglionopathy (AAG) is associated with ganglionic acetylcholine receptor (gAChR) antibodies. We describe a similar but distinct series of patients with autoimmune autonomic failure lacking this antibody. METHODS: Retrospective chart review. RESULTS: Six patients presented with subacute autonomic failure, seronegative for gAChR antibodies. Orthostatic hypotension and gastrointestinal complaints were common. Autonomic testing revealed predominant sympathetic failure and no premature pupillary redilation...
February 2018: Clinical Autonomic Research: Official Journal of the Clinical Autonomic Research Society
https://www.readbyqxmd.com/read/29238017/-the-interface-between-the-immune-system-and-autonomic-nervous-system
#9
REVIEW
Shunya Nakane, Akihiro Mukaino, Yukio Ando
  The nervous system and the immune system are two major systems in human body. Although it was revealed these two systems correlated, the control of immune cell dynamics by the nervous system has come to draw a lot of attention at the present time. Recent advances in basic and preclinical science reveal that reflex neural circuits inhibit the production of cytokines and inflammation in several animal models. One well-characterized cytokine-inhibiting mechanism, termed the "inflammatory reflex", is dependent upon vagus nerve stimulation that inhibits cytokine production and attenuates the inflammation...
2017: Nihon Rinshō Men'eki Gakkai Kaishi, Japanese Journal of Clinical Immunology
https://www.readbyqxmd.com/read/29235746/clinical-problem-solving-a-tobacco-merchant-who-can-t-spit
#10
Sandra Benizri, Nancy Agmon-Levin, Noam D Kitrey, Dan Carter, Elinor Goshen, Yehonathan Sharabi
A 47 year old man presented with a combination of dry mouth and lightheadedness while standing. His medical background was unremarkable except for cigarette smoking and hyperlipidemia. Sjögren's syndrome was ruled out, and he was referred for evaluation of orthostatic hypotension, which by then included syncopal episodes and injuries. Additional symptoms included dry eyes, constipation, reduced sweating, and erectile dysfunction. After excluding medications and structural cardiac abnormalities as causes of orthostatic hypotension, a clinical autonomic evaluation was performed...
December 2017: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/29130498/peripheral-nerve-ultrasound-in-friedreich-ataxia
#11
Eoin Mulroy, Luciana Pelosi, Ruth Leadbetter, Purwa Joshi, Miriam Rodrigues, Stuart Mossman, Dean Kilfoyle, Richard Roxburgh
INTRODUCTION: Sensory impairment in Friedreich ataxia (FRDA) is generally accepted as being due to a ganglionopathy. The degree of contribution from axonal pathology remains a matter of debate. Nerve ultrasound may be able to differentiate these processes. METHODS: The ultrasound cross-sectional area of median, ulnar, tibial, and sural nerves of 8 patients with FRDA was compared with 8 age- and gender-matched healthy controls and with reference values in our population...
November 11, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/29021436/multiple-myeloma-presenting-with-autoimmune-autonomic-ganglionopathy
#12
Yoshiki Nakae, Mizuki Hyuga, Yuta Terada, Wataru Kishimoto, Akiko Fukunaga, Sumie Tabata, Yoshitomo Maesako, Kenichi Komatsu, Osamu Higuchi, Toshinari Nakane, Nobuyoshi Arima
Autoimmune autonomic ganglionopathy is an autonomic disorder that occurs as a symptom of paraneoplastic neurological syndrome. To date, there have been no reports on multiple myeloma with autoimmune autonomic ganglionopathy. A 37-year-old Japanese woman suffered from orthostatic hypotension was diagnosed with multiple myeloma (IgG kappa type), and a serological examination revealed the presence of anti-ganglionic nicotinic acetylcholine receptor (anti-gAChR) antibodies. She was treated for multiple myeloma, as a result, the autonomic disturbance improved and her anti-gAChR antibody titer decreased to undetectable levels, despite the fact that she only achieved a partial remission of multiple myeloma...
December 15, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28968369/sensory-polyneuropathies
#13
Kelly Graham Gwathmey
PURPOSE OF REVIEW: This article describes the methods of diagnosis and management of the sensory-predominant polyneuropathies. To simplify the approach to this category of patients, sensory-predominant polyneuropathies are divided broadly into either small fiber (or pain-predominant) neuropathies and large fiber (or ataxia-predominant) neuropathies, of which the sensory neuronopathies (dorsal root ganglionopathies) are highlighted. RECENT FINDINGS: Physicians can now easily perform skin biopsies in their offices, allowing access to the gold standard pathologic diagnostic tool for small fiber neuropathies...
October 2017: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/28904462/clinical-spectrum-therapeutic-outcomes-and-prognostic-predictors-in-sjogren-s-syndrome-associated-neuropathy
#14
Ajith Sivadasan, Karthik Muthusamy, Bimal Patel, Rohit Ninan Benjamin, A T Prabhakar, Vivek Mathew, Sanjith Aaron, Mathew Alexander
OBJECTIVES: There are limited data regarding long-term follow-up and therapeutic outcomes in Sjogren's syndrome (SS)-associated peripheral neuropathy. In this study, we aim to study the clinical, electrophysiological spectrum and therapeutic responses among the different subtypes of SS-associated neuropathy. The predictors of suboptimal treatment response will be identified. METHODS: The study included a retrospective cohort of patients with SS-associated neuropathy between January 2012 and November 2015...
July 2017: Annals of Indian Academy of Neurology
https://www.readbyqxmd.com/read/28886934/transglutaminase-6-antibodies-in-gluten-neuropathy
#15
Panagiotis Zis, Dasappaiah Ganesh Rao, Ptolemaios Georgios Sarrigiannis, Pascale Aeschlimann, Daniel P Aeschlimann, David Sanders, Richard A Grünewald, Marios Hadjivassiliou
BACKGROUND: TG6 antibodies have been shown to be a marker of gluten ataxia but their presence in the context of other neurological manifestations of gluten sensitivity has not been explored. We investigated the presence of TG6 antibodies in gluten neuropathy (GN), defined as as an otherwise idiopathic peripheral neuropathy associated with serological markers of gluten sensitivity (one or more of antigliadin IgG and/or IgA, endomysial and transglutaminase-2 antibodies). METHODS: This was a cross-sectional study conducted at the Sheffield Institute of Gluten Related Diseases, Royal Hallamshire Hospital, Sheffield, UK...
November 2017: Digestive and Liver Disease
https://www.readbyqxmd.com/read/28837658/dorsal-root-ganglia-hypertrophy-as-in-vivo-correlate-of-oxaliplatin-induced-polyneuropathy
#16
Leonidas Apostolidis, Daniel Schwarz, Annie Xia, Markus Weiler, Andreas Heckel, Tim Godel, Sabine Heiland, Heinz-Peter Schlemmer, Dirk Jäger, Martin Bendszus, Philipp Bäumer
PURPOSE: To investigate in vivo morphological and functional correlates of oxaliplatin-induced peripheral neuropathy (OXA-PNP) by magnetic resonance neurography (MRN). METHODS: Twenty patients (7 female, 13 male, 58.9±10.0 years) with mild to moderate OXA-PNP and 20 matched controls (8 female, 12 male, 55.7±15.6 years) were prospectively enrolled. All patients underwent a detailed neurophysiological examination prior to neuroimaging. A standardized imaging protocol at 3...
2017: PloS One
https://www.readbyqxmd.com/read/28740856/opsoclonus-myoclonus-syndrome-during-rituximab-treatment-for-autoimmune-autonomic-ganglionopathy
#17
Oana M Dumitrascu, Andrew McKeon, Leslie Zuniga, Marie F Grill, Brent P Goodman
No abstract text is available yet for this article.
September 2017: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/28620980/case-of-autoimmune-autonomic-ganglionopathy-manifesting-anhidrosis
#18
Asuka Yoshifuku, Koichi Yoneda, Yusuke Sakiyama, Osamu Higuchi, Shunya Nakane, Takuro Kanekura
Autoimmune autonomic ganglionopathy (AAG), clinically characterized by gastrointestinal dysmotility, orthostatic hypotension and tonic pupils, is an idiopathic acquired disorder of the autonomic nervous system elicited by antibodies against ganglionic acetylcholine receptor (gAChR). We encountered a 60-year-old man who presented with severe anhidrosis, difficulty in thermoregulation, orthostatic hypotension, gastrointestinal dysmotility, tonic pupils and ptosis. Histologically, an anhidrotic skin sample was normal...
June 16, 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/28507261/pure-sensory-ganglionopathy-as-the-first-sign-of-relapse-in-non-hodgkin-lymphoma
#19
Michael Hossack, Jeffrey McClean
No abstract text is available yet for this article.
May 16, 2017: Neurology
https://www.readbyqxmd.com/read/28319594/long-term-cardiovascular-autonomic-and-clinical-changes-after-immunoglobulin-g-immunoadsorption-therapy-in-autoimmune-autonomic-ganglionopathy
#20
Franca Barbic, Franca Dipaola, Francesca Andreetta, Enrico Brunetta, Laura Dalla Vecchia, Renato Mantegazza, Raffaello Furlan, Carlo Antozzi
: A 63-year-old male was diagnosed with autoimmune autonomic ganglionopathy based on the finding of plasma antibodies to the nicotinic acetylcholine receptor (nAChR) of autonomic ganglia. He complained of mouth and eye dryness, dysphagia, severe constipation, erectile dysfunction, urgency, frequent urination, habitual orthostatic syncope and presyncope. A remarkable symptomatic orthostatic hypotension without changes in heart rate was present. We here describe the 3-year time course of the changes in spectral indices of cardiovascular autonomic control LF/HF and LFSAP, dysautonomia symptoms intensity and anti-nAChR antibodies following repetitive selective immunoadsorptions...
July 2017: Journal of Hypertension
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