Autoimmune and paraneoplastic channelopathies |
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Authors: | Steven Vernino |
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Institution: | 1. Department of Neurology, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd., 75390-9036, Dallas, TX
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Abstract: | Thirty years ago, antibodies against the muscle acetylcholine receptor (AChR) were recognized as the cause of myasthenia gravis.
Since then, there has been great interest in identifying other neurological disorders associated with auto-antibodies. Several
other antibody-mediated neuromuscular disorders have been identified, each associated with an antibody against a ligand- or
voltage-gated ion channel. The Lambert-Eaton syndrome is caused by antibodies against voltage-gated calcium channels and often
occurs in patients with small cell lung cancer. Acquired neuromyotonia is caused by voltage-gated potassium channel antibodies,
and autoimmune autonomic ganglionopathy is caused by antibodies against the neuronal AChR in autonomic ganglia. There is good
evidence that antibodies in these disorders cause changes in synaptic function r neuronal excitability by directly inhibiting
ion channel function. More recently, studies have identified ion channel antibodies in patients with certain CNS disorders,
such as steroid-responsive encephalitis and paraneoplastic cerebellar ataxia. It remains unclear if antibodies can gain access
to the CNS and directly cause ion channel dysfunction. Treatment of autoimmune channelopathies includes drugs that help restore
normal neuronal function and treatments to remove pathogenic antibodies (plasma exchange) or modulate the immune response
(steroids or immunosuppressants). These disabling neurological disorders may be dramatically responsive to immunomodulatory
therapy. Future studies will likely lead to identification of other ion channel antibodies and other autoimmune channelopathies. |
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