Recent advances in autoimmune autonomic ganglionopathy.
OBJECTIVE
This review highlights recent advances in our understanding of autoimmune autonomic ganglionopathy (AAG) including serological diagnosis, clinical observations, pathophysiology, and treatment.


RESULTS
Classical AAG is a subacute disorder associated with antibodies against ganglionic acetylcholine receptors. Recent observations demonstrate that these antibodies cause a specific inhibition of synaptic transmission in autonomic ganglia and antibody levels correlate in a predictable way with severity of autonomic failure. Ganglionic acetylcholine receptor antibodies can be associated with other neurological disorders including chronic or restricted forms of autonomic dysfunction and with neurological or paraneoplastic disorders unrelated to the autonomic nervous system. Treatment of AAG is based on case reports. It is clear that AAG patients respond to immunomodulatory treatments but combination immunotherapy may be required to achieve a clinical response. Recently, additional clinical observations indicate that AAG patients may also show signs of treatment-responsive cognitive impairment.


CONCLUSIONS
The pathophysiology and varied clinical presentation of AAG are becoming more clear. AAG is a treatable antibody-mediated disorder of autonomic ganglionic synaptic transmission. Future studies will continue to define the optimal diagnostic and therapeutic approach to autoimmune autonomic disorders.
Winston
Nicole
N
Department of Neurology, UT Southwestern Medical Center, Dallas, Texas 75390-9036, USA.
Vernino
Steven
S
D001323:Autoantibodies / Q000276:immunology; D001324:Autoantigens / Q000276:immunology; D020274:Autoimmune Diseases of the Nervous System / Q000276:immunology* / Q000503:physiopathology* / Q000628:therapy; D005725:Ganglia, Autonomic / Q000276:immunology* / Q000503:physiopathology*; D006801:Humans; D011950:Receptors, Cholinergic / Q000276:immunology
D001323:Autoantibodies; D001324:Autoantigens; D011950:Receptors, Cholinergic