TY - JOUR
T1 - Autoimmune autonomic failure
AU - Muppidi, Srikanth
AU - Vernino, Steven
PY - 2013
Y1 - 2013
N2 - 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. These antibodies cause a similar phenotype of autonomic failure in animal models indicating that an antibody-mediated functional impairment of ganglionic transmission is the underlying etiology. Decrease in antibody levels correlates with clinical improvement. Patients may respond to immunomodulatory therapies such as prednisone, intravenous immunoglobulin, plasma exchange, and oral immunosuppressants. A combination treatment is often required as well as symptomatic therapy.
AB - 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. These antibodies cause a similar phenotype of autonomic failure in animal models indicating that an antibody-mediated functional impairment of ganglionic transmission is the underlying etiology. Decrease in antibody levels correlates with clinical improvement. Patients may respond to immunomodulatory therapies such as prednisone, intravenous immunoglobulin, plasma exchange, and oral immunosuppressants. A combination treatment is often required as well as symptomatic therapy.
KW - Autonomic failure
KW - Autonomic function testing
KW - Dysautonomia
KW - Ganglionic acetylcholine receptor antibody
KW - Immunotherapy
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U2 - 10.1016/B978-0-444-53491-0.00025-0
DO - 10.1016/B978-0-444-53491-0.00025-0
M3 - Article
C2 - 24095135
AN - SCOPUS:84884966673
VL - 117
SP - 321
EP - 327
JO - Handbook of clinical neurology / edited by P.J. Vinken and G.W. Bruyn
JF - Handbook of clinical neurology / edited by P.J. Vinken and G.W. Bruyn
SN - 0072-9752
ER -