Autoimmune autonomic ganglionopathy
Autoimmune autonomic ganglionopathy is a type of immune-mediated autonomic failure that is associated with antibodies against the ganglionic nicotinic acetylcholine receptor present in sympathetic, parasympathetic, and enteric ganglia. Typical symptoms include gastrointestinal dysmotility, orthostatic hypotension, and tonic pupils. Many cases have a sudden onset, but others worsen over time, resembling degenerative forms of autonomic dysfunction. For milder cases, supportive treatment is used to manage symptoms. Plasma exchange, intravenous immunoglobulin, corticosteroids, or immunosuppression have been used successfully to treat more severe cases.
| Autoimmune autonomic ganglionopathy | |
|---|---|
| Other names | Autoimmune autonomic neuropathy, Acute pandysautonomia |
| Functions of the autonomic nervous system. | |
| Specialty | Neurology |
| Symptoms | Gastrointestinal dysmotility, orthostatic hypotension, and tonic pupils. |
| Complications | Weight loss. |
| Diagnostic method | Clinical criteria and serum ganglionic neuronal nicotinic AChR antibodies. |
| Differential diagnosis | Paraneoplastic syndrome, Guillain-Barré syndrome, diabetes, amyloidosis, Sjogren's syndrome, and Morvan syndrome. |
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