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1. Chronic inflammatory demyelinating polyneuropathy

The lumbar puncture shows elevated CSF protein and the nerve conduction velocity studies showed no nerve conduction to the right hemidiaphragm. Of the choices listed, the clinical and neurologic studies are most consistent with chronic inflammatory demyelinating polyneuropathy (CIDP). CIDP is an acquired immune-mediated inflammatory disorder sometimes called chronic relapsing polyneuropathy (CRP) or chronic inflammatory demyelinating polyradiculoneuropathy (1). CIDP is closely related to Guillain-Barré syndrome and acute inflammatory demyelinating polyneuropathy, and it is considered the chronic counterpart of that acute disease.

The diagnostic criteria are listed in table 1 (2).

The patient met the general criteria for CIDP. Phrenic nerve involvement has been reported with CIDP but is rare (3-5). First-line treatment for CIDP includes corticosteroids, plasmapheresis (plasma exchange), and/or intravenous immunoglobulin (IVIG) (1).

Our patient is improving slowly. She is tolerating BiPAP and is receiving IVIG and corticosteroids. She is undergoing occupational and physical therapy with plans to continue therapy at an acute rehabilitation hospital.

References

  1. Dalakas MC. Advances in the diagnosis, pathogenesis and treatment of CIDP. Nat Rev Neurol. 2011;7(9):507-17. [CrossRef] [PubMed]
  2. Saperstein DS, Katz JS, Amato AA, Barohn RJ. Clinical spectrum of chronic acquired demyelinating polyneuropathies. Muscle Nerve. 2001;24(3):311-24. [CrossRef] [PubMed]
  3. Phrenic nerve palsy as a feature of chronic inflammatory demyelinating polyradiculoneuropathy. Stojkovic T, De Seze J, Hurtevent JF, Fourrier F, Vermersch P. Muscle Nerve. 2003;27(4):497-9. [CrossRef] [PubMed]
  4. Kawakami T, Sekijima Y, Tokuda T, Yamazaki M, Yanagisawa N. A case of chronic inflammatory demyelinating polyradiculoneuropathy complicated by phrenic nerve palsy. Rinsho Shinkeigaku. 1998;38(2):118-21. [PubMed]
  5. Kimber TE, Orrell RW, King RH, Ginsberg L. Pathological findings in a patient with ventilatory failure and chronic inflammatory demyelinating polyneuropathy. J Peripher Nerv Syst. 2003;8(1):13-6. [CrossRef] [PubMed]

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