Autoimmune autonomic ganglionopathy (AAG) presents as severe generalized sympathetic
and parasympathetic autonomic failure. This disorder is also known as acute pandysautonomia
or idiopathic subacute autonomic neuropathy. The most disabling manifestations are
orthostatic hypotension (OH) and gastrointestinal dysmotility. An autoimmune pathogenesis
was suggested by the subacute onset, frequent association with antecedent viral illness,
occasional association with cancer or other autoimmune disorders, and spontaneous
recovery in some patients. Many cases have a rapid onset, but others have a chronic
progressive course. Clinical and experimental observations demonstrate that AAG is
an antibody-mediated disorder. (1) AAG patients have high levels of serum antibodies
specific for the neuronal AChR in autonomic ganglia. This ganglionic AChR mediates
fast synaptic transmission in all autonomic ganglia. Ganglionic AChR antibodies found
in AAG patients are distinct from muscle AChR antibodies found in patients with MG.
(2) Ganglionic AChR antibody levels correlate with severity of autonomic deficits.
Patients with high antibody levels have a combination of OH, dry eyes and dry mouth,
Adies pupil, gastroparesis, and neurogenic bladder. (3) Induction of ganglionic AChR
antibodies in rabbits by immunization leads to experimental AAG which reproduces many
of the cardinal features of the human disease. (4) Passive transfer of ganglionic
AChR antibodies to mice causes transient autonomic failure. In electrophysiological
studies, ganglionic AChR antibodies produce a reduction in ganglionic AChR current
in cultured neuroblastoma cells and inhibit synaptic transmission in isolated autonomic
ganglia. AAG is an antibody-mediated channelopathy that produces a severe, but treatable,
form of autonomic failure.
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© 2015 Published by Elsevier Inc.