Chronic inflammatory demyelinating polyradiculoneuropathy

Pioneering, combined antibody testing yields unparalleled insights

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare, oftentimes severe immune-mediated neurological disorder involving peripheral nerve and nerve root inflammation leading to destruction of the protective myelin sheath covering nerve fibers. Frequently misdiagnosed, CIDP in most cases is highly responsive to first-line immunotherapy treatments, such as intravenous immunoglobin (IVIG). However, a subset of individuals with specific paranodal antibodies do not respond as well to these therapies.

Among patients affected by CIDP, the presence of neurofascin 155 (NF155) IgG4 and contactin-1 (CNTN1) IgG indicates a unique disease etiology with antibodies targeting the paranode. These patients have relatively poor treatment response to IVIG. Mayo Clinic Laboratories has developed and validated a unique approach to test for these antibodies. This assay combination is available exclusively at Mayo Clinic Laboratories.

CIDP | Chronic Inflammatory Demyelinating Polyradiculoneuropathy/Nodopathy Evaluation, Serum

Advantages

  • Novel testing available only at Mayo Clinic Laboratories.
  • Enables improved diagnostic accuracy through the use of live cell flow cytometry.
  • Offers increased physician confidence.
  • Assists in guiding treatment selection.  

When to consider testing

  • In the event of inconclusive electrodiagnostic studies.
  • To confirm diagnosis of CIDP.
  • To pinpoint phenotype to guide treatment.

Distinguishing NF155-associated CIDP from CNTN1-associated CIDP

While most CIDP patients present similarly, certain disease associations can help differentiate between disease variants.

  • NF155 IgG4-seropositive patients can present with tumors, proprioceptive loss, and distal weakness.
  • Some CNTN1 patients develop kidney disease and membranous glomerular nephropathy.
  • CNTN1 patients can also develop chronic immune sensory polyneuropathy (CISP), a sensory predominant subvariant of CIDP in which just the sensory nerve roots are affected.

A Test in Focus

Divyanshu (Div) Dubey, M.B.B.S., describes Mayo Clinic Laboratories' new diagnostic test for CIDP, or chronic inflammatory demyelinating polyneuropathy. The new test detects two antibodies — NF155 and CNTN1 — to enhance diagnosis and guide treatment decisions. Often misdiagnosed, CIDP is treatable if detected early.

Learn more about how to order this evaluation at your institution.

References

Querol L, Nogales-Gadea G, Rojas-Garcia R, et al. Antibodies to contactin-1 in chronic inflammatory demyelinating polyneuropathy. Ann Neurol. 2013 Mar;73(3):370-80. doi:10.1002/ana.23794. Epub 2012 Dec 31. PMID: 23280477.

Querol L, Nogales-Gadea G, Rojas-Garcia R, et al. Neurofascin IgG4 antibodies in CIDP associate with disabling tremor and poor response to IVIg. Neurology. 2014 Mar 11;82(10):879-86. doi:10.1212/WNL.0000000000000205. Epub 2014 Feb 12. PMID: 24523485; PMCID: PMC3959751.

Ng JK, Malotka J, Kawakami N, et al. Neurofascin as a target for autoantibodies in peripheral neuropathies. Neurology. 2012 Dec 4;79(23):2241-8. doi:10.1212/WNL.0b013e31827689ad. Epub 2012 Oct 24. PMID: 23100406; PMCID: PMC3542349.

Dubey D, Honorat JA, Shelly S. Contactin-1 autoimmunity: Serologic, neurologic, and pathologic correlates. Neurol Neuroimmunol Neuroinflamm. 2020 May 27;7(4):e771. doi:10.1212/NXI.0000000000000771. PMID: 32461352; PMCID: PMC7286654.

Shelly S, Klein CJ, Dyck PJB, et al. Neurofascin-155 immunoglobulin subtypes: clinicopathologic associations and neurologic outcomes. Neurology. 2021 Dec 14;97(24):e2392-e2403. doi:10.1212/WNL.0000000000012932. Epub 2021 Oct 11. PMID: 34635556; PMCID: PMC8673722.