IgM MGUS associated with anti-MAG neuropathy: a single institution experience
- 767 Downloads
Anti-MAG neuropathy is a very rare form of acquired polyneuropathy associated with IgM monoclonal gammopathy of undetermined significance (MGUS). We conducted a retrospective review of 194 consecutive MGUS patients seen at the Penn State Hershey Cancer Institute. We identified six patients among 37 (16 %) with IgM MGUS with anti-MAG neuropathy. Interestingly, an additional patient had anti-MAG neuropathy without MGUS. Common clinical manifestations were numbness and paresthesias of the extremities and gait imbalance. All four patients treated with rituximab and none of the three untreated ones had a subjective improvement of their symptoms. We conclude that all patients with IgM MGUS and neuropathy should be screened for anti-MAG antibodies and, if positive, they should be offered treatment with rituximab.
KeywordsMGUS IgM Myelin-associated glycoprotein Peripheral neuropathy Rituximab
This research received no funding.
Conflict of interest
- 6.Nobile-Orazio E, Manfredini E, Carpo M, Meucci N, Monaco S, Ferrari S, Bonetti B, Cavaletti G, Gemignani F, Durelli L et al (1994) Frequency and clinical correlates of anti-neural IgM antibodies in neuropathy associated with IgM monoclonal gammopathy. Ann Neurol 36:416–424CrossRefPubMedGoogle Scholar
- 9.Ellie E, Vital A, Steck A, Boiron JM, Vital C, Julien J (1996) Neuropathy associated with “benign” anti-myelin-associated glycoprotein IgM gammopathy: clinical, immunological, neurophysiological pathological findings and response to treatment in 33 cases. J Neurol 243:34–43CrossRefPubMedGoogle Scholar
- 12.Chassande B, Leger JM, Younes-Chennoufi AB, Bengoufa D, Maisonobe T, Bouche P, Baumann N (1998) Peripheral neuropathy associated with IgM monoclonal gammopathy: correlations between M-protein antibody activity and clinical/electrophysiological features in 40 cases. Muscle Nerve 21:55–62CrossRefPubMedGoogle Scholar
- 13.Yeung KB, Thomas PK, King RH, Waddy H, Will RG, Hughes RA, Gregson NA, Leibowitz S (1991) The clinical spectrum of peripheral neuropathies associated with benign monoclonal IgM, IgG and IgA paraproteinaemia. Comparative clinical, immunological and nerve biopsy findings. J Neurol 238:383–391CrossRefPubMedGoogle Scholar
- 27.Nasu S, Misawa S, Sekiguchi Y, Shibuya K, Kanai K, Fujimaki Y, Ohmori S, Mitsuma S, Koga S, Kuwabara S (2012) Different neurological and physiological profiles in POEMS syndrome and chronic inflammatory demyelinating polyneuropathy. J Neurol Neurosurg Psychiatry 83:476–479CrossRefPubMedGoogle Scholar
- 28.European Federation of Neurological Societies/Peripheral Nerve Society (2006) Guideline on management of paraproteinemic demyelinating neuropathies. Report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society. J Peripher Nerv Syst 11:9–19CrossRefGoogle Scholar
- 30.Baldini L, Nobile-Orazio E, Guffanti A, Barbieri S, Carpo M, Cro L, Cesana B, Damilano I, Maiolo AT (1994) Peripheral neuropathy in IgM monoclonal gammopathy and Waldenstrom’s macroglobulinemia: a frequent complication in elderly males with low MAG-reactive serum monoclonal component. Am J Hematol 45:25–31CrossRefPubMedGoogle Scholar
- 34.Xu L, Hunter ZR, Yang G, Cao Y, Liu X, Manning R, Tripsas C, Chen J, Patterson CJ, Kluk M, Kanan S, Castillo J, Lindeman N, Treon SP (2014) Detection of MYD88 L265P in peripheral blood of patients with Waldenstrom’s macroglobulinemia and IgM monoclonal gammopathy of undetermined significance. Leukemia 28:1698–1704CrossRefPubMedGoogle Scholar
- 41.Sala E, Robert-Varvat F, Paul S, Camdessanche JP, Antoine JC (2014) Acute neurological worsening after rituximab treatment in patients with anti-MAG neuropathy. J Neurol Sci 345:224-227Google Scholar