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1 Neurology, Mayo Clinic, Rochester, MN, USA
2 Neurology, Mayo Clinic, Rochester, MN, USA; Immunology and Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA
* To whom correspondence should be addressed. E-mail: verns{at}mayo.edu.
Antibodies specific for the neuronal ganglionic nicotinic acetylcholine receptor (nAChR) are found in high titer in serum of patients with subacute autonomic failure. This clinical disorder is known as autoimmune autonomic neuropathy (AAN). Rabbits immunized with a neuronal nAChR 
3 subunit fusion protein produce ganglionic nAChR antibodies and develop autonomic failure (experimental AAN, or EAAN). We used quantitative measures of autonomic function to demonstrate that this animal model of neuronal nAChR autoimmunity recapitulates the cardinal autonomic features of AAN in man. Signs of dysautonomia in the rabbit range from isolated cardiovagal impairment to severe panautonomic failure with fixed mydriasis, gastroparesis, dry eyes, impaired heart rate variability, hypotension and low plasma catecholamines. The severity of autonomic failure correlates with serum antibody levels. Immunohistochemical staining of superior cervical ganglia and myenteric plexus neurons demonstrates intact presynaptic nerve terminals and intact postsynaptic neurons containing cytoplasmic nAChR, but lacking surface nAChR. These findings define the autonomic physiology and histopathology of this novel animal model and support the concept that AAN in man is a disorder of ganglionic cholinergic synaptic transmission caused by ganglionic nAChR antibodies.
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