Paraneoplastic autonomic dysfunction (PAD) may be immune-mediated or nonimmune-mediated. Paraneoplastic disorder should be suspected in patients with progressive autonomic symptoms, such as orthostatic hypotension, dry mouth, urinary retention, and constipation. Occurrence of sleep apnea, pseudomyotonia, myokymia, pseudoobstruction of bowels, or progressive proprioceptive deficit should strengthen this suspicion. The diagnosis is made by the exclusion of other causes of autonomic dysfunction; electrophysiologic studies in patients with neuromyotonia or suspected Lambert-Eaton myasthenic syndrome; and serologic studies indicating the presence of Anti-Hu/antineuronal nuclear antibody-1, enteric neuronal antibodies, and/or ganglionic acetylcholine receptor (AChR) autoantibodies. A proper diagnosis can spare the patients of unnecessary surgeries in cases of pseudoobstruction of the bowels. Treatment may be directed at the tumor, the antibodies, and the symptoms. An early surgical and cytotoxic reduction of the tumor may diminish autonomic dysfunction in some patients. Use of steroids, plasmapheresis, intravenous (IV) immunoglobulin, or immunosuppression with cyclophosphamide to reduce the antibody may provide benefit.
|Original language||English (US)|
|Title of host publication||Primer on the Autonomic Nervous System|
|Subtitle of host publication||Second Edition|
|Number of pages||3|
|State||Published - May 5 2004|
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