Mayo Clinic Laboratories > Neurology > Autoimmune > Neuropathies > Demyelinating neuropathies

Demyelinating neuropathies

Increase confidence through expert-backed testing

Autoimmune demyelinating neuropathies include several progressive conditions — among them chronic inflammatory demyelinating polyneuropathy (CIDP), Guillain-Barre syndrome (GBS), and chronic immune sensory polyradiculopathy (CISP) — that share overlapping motor and/or sensory features with other peripheral neuropathies.

Demyelinating neuropathies Test menu

chronic inflammatory demyelinating polyneuropathy

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. 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. We’ve developed and validated a unique assay combination available exclusively at Mayo Clinic Laboratories.

Key testing

Advantages

  • Evaluates for antibodies directed against NF155 and CNTN1, two common biomarkers associated with CIDP.
  • Combination assay uses a fixed cell-based assay to measure antibodies directed against CNTN1 protein and live cell flow cytometry to identify antibodies directed against NF155, which offers higher clinical specificity than conventional testing methodologies.

Highlights


myelin-associated glycoprotein

The presence of myelin associated glycoprotein (MAG) antibodies is often linked to a rare demyelinating neuropathy with poor treatment response known as DADS neuropathy. In some cases, reaching correct diagnosis through clinical and electrophysiological evaluation alone can be difficult due to overlapping features, or when patient evaluation occurs later in the disease course. Incorporating MAG antibody testing into the diagnostic workup can help identify the correct diagnosis and exclude patients with treatable immune-mediated neuropathies.

Key testing

Advantages

  • Utilizes a semi-quantitative ELISA platform and human MAG antigen with improved specificity to equip physicians with more accurate information.
  • Consider MAG autoantibody testing in the following instances:
    • Inconclusive electrodiagnostic studies.
    • Presence of an IgM monoclonal protein (M-protein) and neuropathy.
    • Suspicion of DADS neuropathy.

Highlights


References
  1. 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.
  2. 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.
  3. 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.
  4. 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.
  5. 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.
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