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Ganglionopathies

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https://www.readbyqxmd.com/read/28904462/clinical-spectrum-therapeutic-outcomes-and-prognostic-predictors-in-sjogren-s-syndrome-associated-neuropathy
#1
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
#2
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...
August 10, 2017: Digestive and Liver Disease
https://www.readbyqxmd.com/read/28837658/dorsal-root-ganglia-hypertrophy-as-in-vivo-correlate-of-oxaliplatin-induced-polyneuropathy
#3
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
#4
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
#5
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
#6
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
#7
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
https://www.readbyqxmd.com/read/28214088/a-case-of-seropositive-autoimmune-autonomic-ganglionopathy-with-diffuse-esophageal-spasm
#8
Nobutoshi Morimoto, Sakuma Takahashi, Tomoki Inaba, Motonori Takamiya, Yasuhiko Kageyama, Mizuki Morimoto, Yoshiaki Takahashi, Hirotake Nishimura, Shunya Nakane, Koji Abe
Autoimmune autonomic ganglionopathy (AAG) is an immune-mediated disorder that leads to various autonomic failures associated with anti-ganglionic acetylcholine receptor antibodies (anti-gAChR-Abs). Diffuse esophageal spasm (DES) is an uncommon esophageal motility disorder. We herein report the case of a 68-year-old woman with DES as a partial symptom of AAG. She presented with chronic esophageal transit failure, constipation, and numbness of the hands and feet, Adie's pupil, thermal hypoalgesia, and decreased deep tendon reflexes...
May 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28182102/a-case-report-of-nonvasculitic-autoimmune-inflammatory-meningoencephalitis-with-sensory-ganglionopathy-a-rare-presentation-of-sj%C3%A3-gren-syndrome
#9
João Peres, Simão Cruz, Rita Oliveira, Luís Santos, Ana Valverde
A 68-year-old Caucasian female was admitted to the emergency department with a progressive history of behavioural symptoms and anxiety followed by visual and auditory hallucinations, forgetfulness, and impaired gait in the previous 3 months. On examination she was psychotic and had a postural and rest tremor of the upper limbs, cogwheel rigidity of the four limbs, retropulsion on standing position, and inability to walk. During the following 2 weeks she developed xerostomia and unilateral parotiditis that improved with steroids...
2017: Case Reports in Neurological Medicine
https://www.readbyqxmd.com/read/28074147/cerebellar-ataxia-and-sensory-ganglionopathy-associated-with-light-chain-myeloma
#10
Panagiotis Zis, Dasappaiah Ganesh Rao, Bart E Wagner, Lucinda Nicholson-Goult, Nigel Hoggard, Marios Hadjivassiliou
BACKGROUND: Cerebellar ataxia with sensory ganglionopathy is a rare neurological combination that can occur in some hereditary ataxias including mitochondrial diseases and in gluten sensitivity. Individually each condition can be a classic paraneoplastic neurological syndrome. We report a patient with this combination who was diagnosed with light-chain myeloma ten years after initial presentation. CASE PRESENTATION: A 65-year-old Caucasian lady was referred to our Ataxia Clinic because of a 6-year history of progressive unsteadiness and a 2-year history of slurred speech...
2017: Cerebellum & Ataxias
https://www.readbyqxmd.com/read/27861227/sensory-ganglionopathy-associated-with-sj%C3%A3-gren-syndrome
#11
LETTER
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
#12
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 that of controls at all sites (P < 0...
July 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/27704495/neuromuscular-diseases
#13
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
#14
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
#15
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
#16
REVIEW
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
#17
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
#18
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
#19
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
#20
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
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