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Immunoglobulin M deposition in cutaneous nerves of anti-myelin-associated glycoprotein polyneuropathy patients correlates with axonal degeneration.
J Neuropathol Exp Neurol. 2009 Feb; 68(2):148-58.JN

Abstract

Anti-myelin-associated glycoprotein (MAG) neuropathy is an antibody-mediated polyneuropathy. We correlated clinical features, immunoglobulin (Ig) M blood levels, IgM deposition and axonal degeneration in skin biopsies of anti-MAG neuropathy patients. By confocal microscopy, IgM deposits were found exclusively within perineurium-enclosed nerves; they were not found on single, non-perineurium-ensheathed myelinated axons. There was a linear correlation between IgM accumulation in nerve fascicles with IgM blood levels but not with anti-MAG antibody titer or disease duration. Axons with specific IgM deposits had signs of axonal damage, including neurofilament disintegration. Nodal structures were intact even at sites where the axons showed pathologic changes. Ultrastructural analysis revealed degeneration of myelinating Schwann cells. Taken together, these findings suggest that in anti-MAG neuropathy patients, IgM deposits are entrapped within cutaneous perineurium-ensheathed nerve bundles where they accumulate in the endoneurial space. High local IgM levels in the endoneurium may be required for IgM deposition on myelin and subsequent axonal injury and degeneration. This study underlines the importance of early, effective anti-B-cell treatments for preventing progression of this neuropathy.

Authors+Show Affiliations

Neurobiology, Department of Biomedicine and Neurology, University Hospital Basel, Basel, Switzerland.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

19151627

Citation

Stalder, Anna K., et al. "Immunoglobulin M Deposition in Cutaneous Nerves of Anti-myelin-associated Glycoprotein Polyneuropathy Patients Correlates With Axonal Degeneration." Journal of Neuropathology and Experimental Neurology, vol. 68, no. 2, 2009, pp. 148-58.
Stalder AK, Erne B, Reimann R, et al. Immunoglobulin M deposition in cutaneous nerves of anti-myelin-associated glycoprotein polyneuropathy patients correlates with axonal degeneration. J Neuropathol Exp Neurol. 2009;68(2):148-58.
Stalder, A. K., Erne, B., Reimann, R., Renaud, S., Fuhr, P., Thomann, S., Arnold, A., Probst, A., Schaeren-Wiemers, N., & Steck, A. J. (2009). Immunoglobulin M deposition in cutaneous nerves of anti-myelin-associated glycoprotein polyneuropathy patients correlates with axonal degeneration. Journal of Neuropathology and Experimental Neurology, 68(2), 148-58. https://doi.org/10.1097/NEN.0b013e3181958187
Stalder AK, et al. Immunoglobulin M Deposition in Cutaneous Nerves of Anti-myelin-associated Glycoprotein Polyneuropathy Patients Correlates With Axonal Degeneration. J Neuropathol Exp Neurol. 2009;68(2):148-58. PubMed PMID: 19151627.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Immunoglobulin M deposition in cutaneous nerves of anti-myelin-associated glycoprotein polyneuropathy patients correlates with axonal degeneration. AU - Stalder,Anna K, AU - Erne,Beat, AU - Reimann,Regina, AU - Renaud,Susanne, AU - Fuhr,Peter, AU - Thomann,Sandra, AU - Arnold,Andreas, AU - Probst,Alphonse, AU - Schaeren-Wiemers,Nicole, AU - Steck,Andreas J, PY - 2009/1/20/entrez PY - 2009/1/20/pubmed PY - 2009/4/10/medline SP - 148 EP - 58 JF - Journal of neuropathology and experimental neurology JO - J. Neuropathol. Exp. Neurol. VL - 68 IS - 2 N2 - Anti-myelin-associated glycoprotein (MAG) neuropathy is an antibody-mediated polyneuropathy. We correlated clinical features, immunoglobulin (Ig) M blood levels, IgM deposition and axonal degeneration in skin biopsies of anti-MAG neuropathy patients. By confocal microscopy, IgM deposits were found exclusively within perineurium-enclosed nerves; they were not found on single, non-perineurium-ensheathed myelinated axons. There was a linear correlation between IgM accumulation in nerve fascicles with IgM blood levels but not with anti-MAG antibody titer or disease duration. Axons with specific IgM deposits had signs of axonal damage, including neurofilament disintegration. Nodal structures were intact even at sites where the axons showed pathologic changes. Ultrastructural analysis revealed degeneration of myelinating Schwann cells. Taken together, these findings suggest that in anti-MAG neuropathy patients, IgM deposits are entrapped within cutaneous perineurium-ensheathed nerve bundles where they accumulate in the endoneurial space. High local IgM levels in the endoneurium may be required for IgM deposition on myelin and subsequent axonal injury and degeneration. This study underlines the importance of early, effective anti-B-cell treatments for preventing progression of this neuropathy. SN - 0022-3069 UR - https://www.unboundmedicine.com/medline/citation/19151627/Immunoglobulin_M_deposition_in_cutaneous_nerves_of_anti_myelin_associated_glycoprotein_polyneuropathy_patients_correlates_with_axonal_degeneration_ L2 - https://academic.oup.com/jnen/article-lookup/doi/10.1097/NEN.0b013e3181958187 DB - PRIME DP - Unbound Medicine ER -