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Autoimmune autonomic ganglionopathy

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
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
Ichiro Kuki, Hisashi Kawawaki, Shin Okazaki, Yuka Hattori, Asako Horino, Osamu Higuchi, Shunya Nakane
Autoimmune autonomic ganglionopathy (AAG) is an acquired immune-mediated disorder that leads to systemic autonomic failure. Autoantibodies to the ganglionic nicotinic acetylcholine receptor (gAChR) are detected in 50% of AAG patients. We report the first pediatric case of AAG presenting with acute encephalitis. The patient was a 13-year-old boy who presented with orthostatic hypotension, followed by rapidly progressing disturbance of consciousness. Cerebrospinal fluid analysis revealed significant pleocytosis and increased neopterin concentration...
June 2016: Brain & Development
Chafic Karam
The author presents a patient with Harlequin and Horner syndromes as part of an autoimmune autonomic ganglionopathy and suggests implication for work-up and management. In general, Harlequin and Horner syndromes are reported to be caused by either a structural lesion of the sympathetic pathway or, when no structural lesion is found, are presumed to be idiopathic. In this paper, a 76 year old man developed a Harlequin and Horner syndromes in the setting of subacute autonomic failure and other systemic features...
January 2016: Autonomic Neuroscience: Basic & Clinical
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
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
Michiaki Koga
No abstract text is available yet for this article.
September 2015: Nihon Rinsho. Japanese Journal of Clinical Medicine
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
Shahryar Tork, Scott R Kelley, Kevin M Tymitz
No abstract text is available yet for this article.
May 2015: American Surgeon
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
Koki Kawanishi, Kosaku Moribata, Jun Kato, Kenya Murata, Kazuhiro Fukatsu, Hidehiko Tamaki, Daisaku Itou, Yuki Wada, Masao Ichinose
A 37-year-old woman who had previously been diagnosed with idiopathic chronic intestinal pseudo-obstruction (CIPO) at another hospital was admitted to our institution with severe abdominal pain. She had a history of several abdominal surgeries to treat ileus at the previous hospital, and contrast-enhanced computed tomography on admission revealed subileus without any apparent causes of obstruction. Total parenteral nutrition, a gastrointestinal prokinetic agent, and opiates reduced persistent pain;however, breakthrough pain continued...
January 2015: Nihon Shokakibyo Gakkai Zasshi, the Japanese Journal of Gastro-enterology
Yuebing Li, Adham Jammoul, Karin Mente, Jianbo Li, Robert W Shields, Steven Vernino, Alexander Rae-Grant
INTRODUCTION: Antibody against the acetylcholine receptor of autonomic ganglia (gAChR-Ab) is implicated in the pathogenesis of autoimmune autonomic ganglionopathy (AAG) and several other disorders. METHODS: This study was a retrospective evaluation of 95 patients positive for gAChR-Ab. RESULTS: Twenty-one (22%) patients had AAG, with a greater median gAChR-Ab level (0.21 nmol/L) and higher percentage (57%) of antibody levels >0.20 nmol/L when compared with the remaining 74 patients without autonomic manifestations (non-AAG group, 0...
September 2015: Muscle & Nerve
Steven K Baker, Beverly M Chow, Steven A Vernino
No abstract text is available yet for this article.
October 2014: Neurology® Neuroimmunology & Neuroinflammation
Eduardo E Benarroch
Central or peripheral neurological disorders can manifest with autonomic failure or autonomic hyperactivity, which may affect the sympathetic, parasympathetic and/or enteric nervous systems. Disorders causing autonomic failure can be classified according to the presence or absence of associated neurological manifestations, such as peripheral neuropathy or parkinsonism, and their temporal profile (acute or subacute, chronic progressive, static, or episodic). A systematic approach allows focused evaluation to detect treatable, potentially disabling or life-threatening conditions...
July 2014: Nature Reviews. Neurology
Masato Asahina
The discovery of acetylcholine was closely related to research on the autonomic nervous system. At the onset of the twentieth century, John Newport Langley (1852-1925), a patriarch of modern autonomic research, classified the autonomic nervous system into the sympathetic, parasympathetic, and enteral systems, proposed the concept of preganglionic and postganglionic autonomic nerves, and suggested the presence of a "receptive substance" allowing the interaction of postganglionic nerve terminals and effector visceral organs...
May 2014: Brain and Nerve, Shinkei Kenkyū No Shinpo
Haruki Koike, Gen Sobue
Autonomic neuropathies may occur primarily or secondarily to various underlying diseases. Primary autonomic neuropathies are divided into pure autonomic neuropathy, autonomic neuropathy with sensory impairment, and autonomic neuropathy with sensory and motor impairment based on the concomitance or absence of sensory or motor dysfunctions. Autoimmune autonomic ganglionopathy refers to a pure autonomic neuropathy, which typically affects both cholinergic and adrenergic functions. About a half of the patients with autoimmune autonomic ganglionopathy are positive for anti-ganglionic acetylcholine receptor antibodies...
2013: Rinshō Shinkeigaku, Clinical Neurology
Shunya Nakane
Autoimmune autonomic ganglionopathy (AAG) is a disorder of isolated autonomic failure associated with antibodies to the nitotinic acetylcholine receptor of the autonomic ganglia resulting in severe autonomic dysfunction. The disorder is associated with and most likely caused by antibodies to gAChR. In this study, we attempted to develop a novel technique to detect antibodies that bind to gAChR. We established a simple in vitro system termed GLIP (gaussia luciferase-reporter immunoprecipitation), which can detect protein-protein interactions with high sensitivity and using no radioisotope...
2013: Rinshō Shinkeigaku, Clinical Neurology
Srikanth Muppidi, Steven Vernino
Autoimmune autonomic ganglionopathy is a syndrome of panautonomic failure caused by antibodies to ganglionic acetylcholine receptors. The clinical syndrome is characterized by significant postural hypotension, diffuse cholinergic and adrenergic impairment, gastrointestinal dysmotility, urinary retention, and pupillary dysfunction. While acute to subacute onset of disease is commonly seen, chronic, slowly progressive variants have been described. Serological testing for ganglionic acetylcholine receptor antibodies helps confirm the diagnosis...
2013: Handbook of Clinical Neurology
Haruki Koike, Gen Sobue
Recent progress in serological screening of paraneoplastic antibodies and in diagnostic imaging techniques to detect malignancies has enabled a broadening of the concept of paraneoplastic neurological syndromes by integrating nonclassic clinical features. The peripheral nervous system is frequently involved in patients with paraneoplastic syndrome and may be seen alone or in combination with involvement of other areas of the nervous system. Destruction of dorsal root ganglion cells due to lymphocytic infiltration, especially with CD8-positive cytotoxic T cells, has been postulated to mediate the classic syndrome of subacute sensory neuronopathy...
2013: Handbook of Clinical Neurology
Michiaki Koga
Acute pandysautonomia is an idiopathic, acute or subacute autonomic neuropathy, which diffusely affects pre- and post-synaptic, and sympathetic and parasympathetic nerves. The recent discovery of serum autoantibodies against the nicotinic acethylcholine receptor (nAChR) on autonomic ganglia has led to a better understanding of its pathogenesis as well as the emergence of a new disease entity named autoimmune autonomic ganglionopathy (AAG). Based on the detection of these antibodies in various dysautonomic conditions, AAG is considered a broad-spectrum disease entity that includes acute pandysautonomia as well as secondary autonomic neuropathy, restricted forms of dysautonomia (postural tachycardia syndrome and chronic intestinal pseudoobstruction), and chronic dysautonomia, mimicking pure autonomic failure...
April 2013: Brain and Nerve, Shinkei Kenkyū No Shinpo
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