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Washington University Experience | PERIPHERAL NEUROPATHY | 7B CIDP | 21A0 Case 21 History
Case 21 History ---- The patient is a 36-year-old man with two years of progressive deficits rendering him quadriplegic with ptosis, ophthalmoplegia, respiratory failure, and dysautonomia. Electrodiagnostic testing evolved from an acquired demyelinating polyneuropathy to a severe demyelinating polyneuropathy with secondary axon loss. He has prior imaging showing enhancement of the cauda equina and cranial nerves. CSF analysis showed cytoalbuminologic dissociation (CSF protein of 179 mg/dL soon after onset which then climbed to a more recent value of 508 mg/dL). A demyelinating neuropathy autoantibody panel was negative. VEGF values are normal on multiple checks. The patient hasn't responded to q 2 week IVIg, mycophenolate mofetil, and prednisone. He has undergone extensive evaluation for a hematologic neoplasm, including POEMS syndrome, and other paraneoplastic disorders. There has been some recent improvement in the cranial neuropathies with plasma exchange and rituximab. The working diagnosis is an unusual form of CIDP.
