Author: Peter Bakes, M.D.
- Rare disease with 1-2 patients out of 100,000. About 60% of patients report a preceding diarrheal illness and classically presents with an ascending motor weakness.
- Pathophysiology is likely due to molecular mimicry where the immune system creates antibodies against a pathogen (C. jejuni ) which appears similar to the myelin of peripheral nerves resulting in autoimmune demyelination.
- Diagnosis is made by clinical presentation +/- a spinal tap with a characteristic high protein count but without cells.
- Treatment is IVIG or plasmapharesis. It is important to monitor respiratory function because about 15% of patients progress to respiratory failure.
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