| 词条 | Autoimmune autonomic ganglionopathy |
| 释义 |
}}{{Infobox medical condition (new) | name = Autoimmune autonomic ganglionopathy | synonyms = | image = | alt = | caption = | pronounce = | field = Neurology | symptoms = | complications = | onset = | duration = | types = | causes = | risks = | diagnosis = | differential = | prevention = | treatment = | medication = | prognosis = | frequency = | deaths = }} Autoimmune autonomic ganglionopathy (AAG) is an extremely rare form of dysautonomia in which the patients immune system produces ganglionic AChR antibodies, inhibiting ganglionic AChR currents and impairing transmission in autonomic ganglia.[1] Approximately 100 Americans are diagnosed with AAG each year. Symptoms onset can be acute, subacute or gradual. Signs and symptomsAlthough symptoms of AAG can range from patient to patient, hallmark symptoms include:
CausesThe cause is generally either paraneoplastic syndrome or idiopathic. In idiopathic AAG, the body's own immune system damages a receptor in the autonomic ganglia, which is part of a peripheral nerve fibre. If the AAG is paraneoplastic, they have a form of cancer, and their immune system has produced paraneoplastic antibodies in response to the cancer.[2] DiagnosisTraditional autonomic testing is used to aid in the diagnosis of AAG. These tests can include a Tilt Table Test (TTT), thermoregulatory sweat test (TST), quantitative sudomotor autonomic reflex testing (QSART) and various blood panels. Additionally, a blood test showing high levels of the antibody ganglionic nicotenic acetylcholine receptor (gAChr) occur in about 50% of patients with AAG (seropositive AAG). The seronegative patients (those without detectable gAChR levels) are theorized to have one or more different antibodies responsible for the autonomic dysfunction. However, both seropositive and seronegative patients have been seen to respond to the same treatments. A paraneoplastic panel may also be ordered to rule out paraneoplastic syndrome.[3] TreatmentWhere an underlying neoplasm is the cause, treatment of this condition is indicated in order to reduce progression of symptoms. For cases without a known cause, treatment involves suppression of the immune system with corticosteroid treatment, intravenous immunoglobulin, immunosuppressive agents like Rituximab, Cellcept, or Imuran or plasmapheresis.[4] See also
References1. ^{{cite journal|doi=10.1016/j.autneu.2008.09.005|author=Steven Vernino, MD|author2=Steve Hopkins|author3=Zhengbei Wang, MD|last-author-amp=yes|journal=Autonomic Neuroscience|volume=146|issue=1–2|pages=3–7|year=2009|title=Autonomic ganglia, acetylcholine receptor antibodies, and autoimmune ganglionopathy|pmid=18951069|pmc=2677210}} 2. ^1 {{cite journal|doi=10.1016/j.autneu.2008.10.022|author1=Paola Sandroni |author2=Phillip A. Low |lastauthoramp=yes |journal=Autonomic Neuroscience|volume=146|issue=1–2|pages=13–17|year=2009|title=Other Autonomic Neuropathies Associated with Ganglionic Antibody|pmid=19058765|pmc=2671239}} 3. ^{{cite book| chapter-url=https://mayoclinic.pure.elsevier.com/en/publications/autoimmune-autonomic-ganglionopathy | chapter=Autoimmune Autonomic Ganglionopathy| doi=10.1016/B978-0-12-386525-0.00100-1| title=Primer on the Autonomic Nervous System| pages=489–492| year=2012| last1=Vernino| first1=Steven| last2=Low| first2=Phillip A.| isbn=9780123865250| citeseerx=10.1.1.657.2841}} 4. ^{{cite journal|doi=10.1001/archneurol.2007.60|pmid=18268189|author=Christopher H. Gibbons, MD, MMSc|author2=Steven A. Vernino, MD|author3=Roy Freeman, MD |lastauthoramp=yes |journal=Arch. Neurol.|volume=65|issue=2|pages=213–217|year=2007|url=http://archneur.jamanetwork.com/article.aspx?articleid=795214|title=Combined Immunomodulatory Therapy in Autoimmune Autonomic Ganglionopathy}} External links{{Medical resources| DiseasesDB = | ICD10 = G90.9, G13.0 | ICD9 = | ICDO = | OMIM = | MedlinePlus = | eMedicineSubj = | eMedicineTopic = | MeshID = }}
4 : Channelopathies|Peripheral nervous system disorders|Autonomic ganglia|Autoimmune diseases |
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