Paraproteinemia and neuropathy.
Monoclonal gammopathy
POEMS syndrome
Paraprotein
Plasma cell dyscrasia
Uncompacted myelin lamellae
Widely spaced myelin
Journal
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
ISSN: 1590-3478
Titre abrégé: Neurol Sci
Pays: Italy
ID NLM: 100959175
Informations de publication
Date de publication:
Nov 2021
Nov 2021
Historique:
received:
16
06
2021
accepted:
28
08
2021
pubmed:
17
9
2021
medline:
21
10
2021
entrez:
16
9
2021
Statut:
ppublish
Résumé
Paraproteinemia is associated with different peripheral neuropathies. The major causes of neuropathy correlated with paraproteinemia are the deposition of immunoglobulin in the myelin, represented by anti-myelin-associated glycoprotein (MAG) neuropathy; deposition of immunoglobulin or its fragment in the interstitium, represented by immunoglobulin light chain amyloidosis (AL amyloidosis); and paraneoplastic mechanisms that cannot be solely attributed to the deposition of immunoglobulin or its fragment, represented by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin change (POEMS) syndrome. Patients with anti-MAG neuropathy and POEMS syndrome present with slowing of nerve conduction parameters. This characteristic fulfills the electrodiagnostic criteria for chronic inflammatory demyelinating polyneuropathy (CIDP) defined by the European Academy of Neurology and Peripheral Nerve Society (EAN/PNS). Although direct damage caused by the deposition of amyloid can induce axonal damage in AL amyloidosis, some patients with this condition have features fulfilling the EAN/PNS electrodiagnostic criteria for CIDP. Conventional immunotherapies for CIDP, such as steroids, intravenous immunoglobulin, and plasma exchange, offer no or only minimal-to-modest benefit. Although rituximab can reduce the level of circulating autoantibodies, it may only be effective in some patients with anti-MAG neuropathy. Drugs including melphalan, thalidomide, lenalidomide, and bortezomib for POEMS syndrome and those including melphalan, thalidomide, lenalidomide, pomalidomide, bortezomib, ixazomib, and daratumumab for AL amyloidosis are considered. Since there will be more therapeutic options in the future, thereby enabling appropriate treatments for individual neuropathies, there is an increasing need for early diagnosis.
Identifiants
pubmed: 34529193
doi: 10.1007/s10072-021-05583-7
pii: 10.1007/s10072-021-05583-7
doi:
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
4489-4501Subventions
Organisme : the ministry of health, labour and welfare of japan
ID : 20FC1030
Informations de copyright
© 2021. Fondazione Società Italiana di Neurologia.
Références
Glavey SV, Leung N (2016) Monoclonal gammopathy: the good, the bad and the ugly. Blood Rev 30(3):223–231
pubmed: 26732417
doi: 10.1016/j.blre.2015.12.001
pmcid: 26732417
Kyle RA, Larson DR, Therneau TM, Dispenzieri A, Kumar S, Cerhan JR, Rajkumar SV (2018) Long-term follow-up of monoclonal gammopathy of undetermined significance. N Engl J Med 378(3):241–249
pubmed: 29342381
pmcid: 5852672
doi: 10.1056/NEJMoa1709974
Chaudhry HM, Mauermann ML, Rajkumar SV (2017) Monoclonal gammopathy-associated peripheral neuropathy: diagnosis and management. Mayo Clin Proc 92(5):838–850
pubmed: 28473042
doi: 10.1016/j.mayocp.2017.02.003
pmcid: 28473042
Saleun JP, Vicariot M, Deroff P, Morin JF (1982) Monoclonal gammopathies in the adult population of Finistère. France J Clin Pathol 35(1):63–68
pubmed: 6801095
doi: 10.1136/jcp.35.1.63
pmcid: 6801095
Crawford J, Eye MK, Cohen HJ (1987) Evaluation of monoclonal gammopathies in the “well” elderly. Am J Med 82(1):39–45
pubmed: 3492143
doi: 10.1016/0002-9343(87)90375-5
pmcid: 3492143
Kyle RA, Therneau TM, Rajkumar SV, Larson DR, Plevak MF, Offord JR, Dispenzieri A, Katzmann JA, Melton LJ 3rd (2006) Prevalence of monoclonal gammopathy of undetermined significance. N Engl J Med 354(13):1362–1369
pubmed: 16571879
doi: 10.1056/NEJMoa054494
pmcid: 16571879
Vallat JM, Duchesne M, Corcia P, Richard L, Ghorab K, Magy L, Mathis S (2021) The wide spectrum of pathophysiologic mechanisms of paraproteinemic neuropathy. Neurology 96(5):214–225
pubmed: 33277411
pmcid: 33277411
Kelly JJ Jr, Kyle RA, O’Brien PC, Dyck PJ (1981) Prevalence of monoclonal protein in peripheral neuropathy. Neurology 31(11):1480–1483
pubmed: 6273767
doi: 10.1212/WNL.31.11.1480
pmcid: 6273767
Tomita M, Koike H, Kawagashira Y, Iijima M, Adachi H, Taguchi J, Abe T, Sako K, Tsuji Y, Nakagawa M, Kanda F, Takeda F, Sugawara M, Toyoshima I, Asano N, Sobue G (2013) Clinicopathological features of neuropathy associated with lymphoma. Brain 136(Pt 8):2563–2578
pubmed: 23884813
doi: 10.1093/brain/awt193
pmcid: 23884813
Latov N (1995) Pathogenesis and therapy of neuropathies associated with monoclonal gammopathies. Ann Neurol 37(Suppl 1):S32-42
pubmed: 8968215
doi: 10.1002/ana.410370705
pmcid: 8968215
Kawagashira Y, Koike H, Tomita M, Morozumi S, Iijima M, Nakamura T, Katsuno M, Tanaka F, Sobue G (2010) Morphological progression of myelin abnormalities in IgM-monoclonal gammopathy of undetermined significance anti-myelin-associated glycoprotein neuropathy. J Neuropathol Exp Neurol 69(11):1143–1157
pubmed: 20940626
doi: 10.1097/NEN.0b013e3181fa44af
pmcid: 20940626
Koike H, Mouri N, Fukami Y, Iijima M, Matsuo K, Yagi N, Saito A, Nakamura H, Takahashi K, Nakae Y, Okada Y, Tanaka F, Sobue G, Katsuno M (2021) Two distinct mechanisms of neuropathy in immunoglobulin light chain (AL) amyloidosis. J Neurol Sci 421:117305
pubmed: 33540321
doi: 10.1016/j.jns.2020.117305
pmcid: 33540321
Koike H, Iijima M, Mori K, Yamamoto M, Hattori N, Watanabe H, Tanaka F, Doyu M, Sobue G (2008) Neuropathic pain correlates with myelinated fibre loss and cytokine profile in POEMS syndrome. J Neurol Neurosurg Psychiatry 79(10):1171–1179
pubmed: 18356256
doi: 10.1136/jnnp.2007.135681
pmcid: 18356256
Ramchandren S, Lewis RA (2009) Monoclonal gammopathy and neuropathy. Curr Opin Neurol 22(5):480–485
pubmed: 19625962
doi: 10.1097/WCO.0b013e32832fd563
pmcid: 19625962
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(3):416–424
pubmed: 8080249
doi: 10.1002/ana.410360313
pmcid: 8080249
Campagnolo M, Zambello R, Nobile-Orazio E, Benedetti L, Marfia GA, Riva N, Castellani F, Bianco M, Salvalaggio A, Garnero M, Ruiz M, Mataluni G, Fazio R, Ermani M, Briani C (2017) IgM MGUS and Waldenstrom-associated anti-MAG neuropathies display similar response to rituximab therapy. J Neurol Neurosurg Psychiatry 88(12):1094–1097
pubmed: 28501820
doi: 10.1136/jnnp-2017-315736
pmcid: 28501820
Svahn J, Petiot P, Antoine JC, Vial C, Delmont E, Viala K, Steck AJ, Magot A, Cauquil C, Zarea A, Echaniz-Laguna A, Iancu Ferfoglia R, Gueguen A, Magy L, Léger JM, Kuntzer T, Ferraud K, Lacour A, Camdessanché JP (2018) Francophone anti-MAG cohort Group. Anti-MAG antibodies in 202 patients: clinicopathological and therapeutic features. J Neurol Neurosurg Psychiatry 89(5):499–505
pubmed: 29070644
doi: 10.1136/jnnp-2017-316715
pmcid: 29070644
Steck AJ, Stalder AK, Renaud S (2006) Anti-myelin-associated glycoprotein neuropathy. Curr Opin Neurol 19(5):458–463
pubmed: 16969155
doi: 10.1097/01.wco.0000245368.36576.0d
pmcid: 16969155
Lunn MP, Crawford TO, Hughes RA, Griffin JW, Sheikh KA (2002) Anti-myelin-associated glycoprotein antibodies alter neurofilament spacing. Brain 125(Pt 4):904–911
pubmed: 11912122
doi: 10.1093/brain/awf072
pmcid: 11912122
Trapp BD, Quarles RH (1982) Presence of the myelin-associated glycoprotein correlates with alterations in the periodicity of peripheral myelin. J Cell Biol 92(3):877–882
pubmed: 6177705
doi: 10.1083/jcb.92.3.877
pmcid: 6177705
Lach B, Rippstein P, Atack D, Afar DE, Gregor A (1993) Immunoelectron microscopic localization of monoclonal IgM antibodies in gammopathy associated with peripheral demyelinative neuropathy. Acta Neuropathol 85(3):298–307
pubmed: 8384775
doi: 10.1007/BF00227726
pmcid: 8384775
Vallat JM, Magy L, Sindou P, Magdelaine C, Cros D (2005) IgG neuropathy: an immunoelectron microscopic study. J Neuropathol Exp Neurol 64(5):386–390
pubmed: 15892295
doi: 10.1093/jnen/64.5.386
pmcid: 15892295
Vallat JM, Tabaraud F, Sindou P, Preux PM, Vandenberghe A, Steck A (2000) Myelin widenings and MGUS-IgA: an immunoelectron microscopic study. Ann Neurol 47(6):808–811
pubmed: 10852548
doi: 10.1002/1531-8249(200006)47:6<808::AID-ANA15>3.0.CO;2-8
pmcid: 10852548
Monaco S, Bonetti B, Ferrari S, Moretto G, Nardelli E, Tedesco F, Mollnes TE, Nobile-Orazio E, Manfredini E, Bonazzi L et al (1990) Complement-mediated demyelination in patients with IgM monoclonal gammopathy and polyneuropathy. N Engl J Med 322(10):649–652
pubmed: 1689461
doi: 10.1056/NEJM199003083221002
pmcid: 1689461
Koike H, Kadoya M, Kaida KI, Ikeda S, Kawagashira Y, Iijima M, Kato D, Ogata H, Yamasaki R, Matsukawa N, Kira JI, Katsuno M, Sobue G (2017) Paranodal dissection in chronic inflammatory demyelinating polyneuropathy with anti-neurofascin-155 and anti-contactin-1 antibodies. J Neurol Neurosurg Psychiatry 88(6):465–473
pubmed: 28073817
doi: 10.1136/jnnp-2016-314895
pmcid: 28073817
Koike H, Katsuno M (2020) Pathophysiology of chronic inflammatory demyelinating polyneuropathy: insights into classification and therapeutic strategy. Neurol Ther 9(2):213–227
pubmed: 32410146
pmcid: 7606443
doi: 10.1007/s40120-020-00190-8
Kawagashira Y, Koike H, Takahashi M, Ohyama K, Iijima M, Katsuno M, Niwa JI, Doyu M, Sobue G (2020) Aberrant expression of nodal and paranodal molecules in neuropathy associated with IgM monoclonal gammopathy with anti-myelin-associated glycoprotein antibodies. J Neuropathol Exp Neurol 79(12):1303–1312
pubmed: 32856086
doi: 10.1093/jnen/nlaa085
pmcid: 32856086
Koike H, Katsuno M (2021) Macrophages and autoantibodies in demyelinating diseases. Cells 10(4):844
pubmed: 33917929
pmcid: 8068327
doi: 10.3390/cells10040844
Koike H, Nishi R, Ikeda S, Kawagashira Y, Iijima M, Katsuno M, Sobue G (2018) Ultrastructural mechanisms of macrophage-induced demyelination in CIDP. Neurology 91(23):1051–1060
pubmed: 30429275
doi: 10.1212/WNL.0000000000006625
pmcid: 30429275
Koike H, Fukami Y, Nishi R, Kawagashira Y, Iijima M, Katsuno M, Sobue G (2020) Ultrastructural mechanisms of macrophage-induced demyelination in Guillain-Barré syndrome. J Neurol Neurosurg Psychiatry 91(6):650–659
pubmed: 32245766
doi: 10.1136/jnnp-2019-322479
pmcid: 32245766
Kawagashira Y, Kondo N, Atsuta N, Iijima M, Koike H, Katsuno M, Tanaka F, Kusunoki S, Sobue G (2010) IgM MGUS anti-MAG neuropathy with predominant muscle weakness and extensive muscle atrophy. Muscle Nerve 42(3):433–435
pubmed: 20665518
doi: 10.1002/mus.21741
pmcid: 20665518
Magy L, Kaboré R, Mathis S, Lebeau P, Ghorab K, Caudie C, Vallat JM. 2015 Heterogeneity of polyneuropathy associated with anti-MAG antibodies. J Immunol Res. 2015:450391.
Van den Bergh PYK, van Doorn PA, Hadden RDM, Avau B, Vankrunkelsven P, Allen JA, Attarian S, Blomkwist PH, Cornblath DR, Eftimov F, Goedee HS, Harbo T, Kuwabara S, Lewis RA, Lunn MP, Nobile-Orazio E, Querol L, Rajabally YA, Sommer C, Topaloglu HA. European Academy of Neurology/Peripheral Nerve Society Guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint Task Force - Second Revision. J Peripher Nerv Syst. in press.
Kaku DA, England JD, Sumner AJ (1994) Distal accentuation of conduction slowing in polyneuropathy associated with antibodies to myelin-associated glycoprotein and sulphated glucuronyl paragloboside. Brain 117(Pt 5):941–947
pubmed: 7953603
doi: 10.1093/brain/117.5.941
pmcid: 7953603
Trojaborg W, Hays AP, van den Berg L, Younger DS, Latov N (1995) Motor conduction parameters in neuropathies associated with anti-MAG antibodies and other types of demyelinating and axonal neuropathies. Muscle Nerve 18(7):730–735
pubmed: 7540258
doi: 10.1002/mus.880180709
pmcid: 7540258
Capasso M, Torrieri F, Di Muzio A, De Angelis MV, Lugaresi A, Uncini A (2002) Can electrophysiology differentiate polyneuropathy with anti-MAG/SGPG antibodies from chronic inflammatory demyelinating polyneuropathy? Clin Neurophysiol 113(3):346–353
pubmed: 11897535
doi: 10.1016/S1388-2457(02)00011-1
pmcid: 11897535
Gondim FA, De Sousa EA, Latov N, Sander HW, Chin RL, Brannagan TH (2007) Anti-MAG/SGPG associated neuropathy does not commonly cause distal nerve temporal dispersion. J Neurol Neurosurg Psychiatry 78(8):902–904
pubmed: 17353253
pmcid: 2117731
doi: 10.1136/jnnp.2006.111930
Nobile-Orazio E, Bianco M, Nozza A (2017) Advances in the treatment of paraproteinemic neuropathy. Curr Treat Options Neurol 19(12):43
pubmed: 29034435
doi: 10.1007/s11940-017-0479-9
pmcid: 29034435
Dalakas MC (2018) Advances in the diagnosis, immunopathogenesis and therapies of IgM-anti-MAG antibody-mediated neuropathies. Ther Adv Neurol Disord 15(11):1756285617746640
Maurer MA, Rakocevic G, Leung CS, Quast I, Lukačišin M, Goebels N, Münz C, Wardemann H, Dalakas M, Lünemann JD (2012) Rituximab induces sustained reduction of pathogenic B cells in patients with peripheral nervous system autoimmunity. J Clin Invest 122(4):1393–1402
pubmed: 22426210
pmcid: 3314454
doi: 10.1172/JCI58743
Dalakas MC, Rakocevic G, Salajegheh M, Dambrosia JM, Hahn AF, Raju R, McElroy B (2009) Placebo-controlled trial of rituximab in IgM anti-myelin-associated glycoprotein antibody demyelinating neuropathy. Ann Neurol 65(3):286–293
pubmed: 19334068
doi: 10.1002/ana.21577
pmcid: 19334068
Léger JM, Viala K, Nicolas G, Créange A, Vallat JM, Pouget J, Clavelou P, Vial C, Steck A, Musset L, Marin B, RIMAG Study Group (2013) (France and Switzerland). Placebo-controlled trial of rituximab in IgM anti-myelin-associated glycoprotein neuropathy. Neurology 80(24):2217–25
pubmed: 23667063
pmcid: 3721095
doi: 10.1212/WNL.0b013e318296e92b
Levine TD, Pestronk A (1999) IgM antibody-related polyneuropathies: B-cell depletion chemotherapy using Rituximab. Neurology 52(8):1701–1704
pubmed: 10331706
doi: 10.1212/WNL.52.8.1701
pmcid: 10331706
Kawagashira Y, Koike H, Ohyama K, Hashimoto R, Iijima M, Adachi H, Katsuno M, Chapman M, Lunn M, Sobue G (2015) Axonal loss influences the response to rituximab treatment in neuropathy associated with IgM monoclonal gammopathy with anti-myelin-associated glycoprotein antibody. J Neurol Sci 348(1–2):67–73
pubmed: 25467141
doi: 10.1016/j.jns.2014.11.006
pmcid: 25467141
Lunn MP, Nobile-Orazio E (2016) Immunotherapy for IgM anti-myelin-associated glycoprotein paraprotein-associated peripheral neuropathies. Cochrane Database Syst Rev 10(10):CD002827
pubmed: 27701752
pmcid: 27701752
Iancu Ferfoglia R, Guimarães-Costa R, Viala K, Musset L, Neil J, Marin B, Léger JM (2016) Long-term efficacy of rituximab in IgM anti-myelin-associated glycoprotein neuropathy: RIMAG follow-up study. J Peripher Nerv Syst 21(1):10–14
pubmed: 26748872
doi: 10.1111/jns.12156
pmcid: 26748872
Nakanishi T, Sobue I, Toyokura Y, Nishitani H, Kuroiwa Y, Satoyoshi E, Tsubaki T, Igata A, Ozaki Y (1984) The Crow-Fukase syndrome: a study of 102 cases in Japan. Neurology 34(6):712–720
pubmed: 6539431
doi: 10.1212/WNL.34.6.712
pmcid: 6539431
Dispenzieri A (2019) POEMS Syndrome: 2019 Update on diagnosis, risk-stratification, and management. Am J Hematol 94(7):812–827
pubmed: 31012139
pmcid: 31012139
Suichi T, Misawa S, Beppu M, Takahashi S, Sekiguchi Y, Shibuya K, Amino H, Tsuneyama A, Suzuki YI, Nakamura K, Sato Y, Kuwabara S (2019) Prevalence, clinical profiles, and prognosis of POEMS syndrome in Japanese nationwide survey. Neurology 93(10):e975–e983
pubmed: 31371568
doi: 10.1212/WNL.0000000000008062
pmcid: 31371568
Suichi T, Misawa S, Sekiguchi Y, Shibuya K, Tsuneyama A, Suzuki YI, Nakamura K, Kano H, Kuwabara S (2020) Treatment response and prognosis of POEMS syndrome coexisting with Castleman disease. J Neurol Sci 413:116771
pubmed: 32172013
doi: 10.1016/j.jns.2020.116771
pmcid: 32172013
Adams D, Said G (1998) Ultrastructural characterisation of the M protein in nerve biopsy of patients with POEMS syndrome. J Neurol Neurosurg Psychiatry 64(6):809–812
pubmed: 9647319
pmcid: 2170108
doi: 10.1136/jnnp.64.6.809
Watanabe O, Arimura K, Kitajima I, Osame M, Maruyama I (1996) Greatly raised vascular endothelial growth factor (VEGF) in POEMS syndrome. Lancet 347(9002):702
pubmed: 8596427
doi: 10.1016/S0140-6736(96)91261-1
pmcid: 8596427
Kanai K, Sawai S, Sogawa K, Mori M, Misawa S, Shibuya K, Isose S, Fujimaki Y, Noto Y, Sekiguchi Y, Nasu S, Nakaseko C, Takano S, Yoshitomi H, Miyazaki M, Nomura F, Kuwabara S (2012) Markedly upregulated serum interleukin-12 as a novel biomarker in POEMS syndrome. Neurology 79(6):575–582
pubmed: 22843279
doi: 10.1212/WNL.0b013e318263c42b
pmcid: 22843279
Sobue G, Doyu M, Watanabe M, Hayashi F, Mitsuma T (1992) Extensive demyelinating changes in the peripheral nerves of Crow-Fukase syndrome: a pathological study of one autopsied case. Acta Neuropathol 84(2):171–177
pubmed: 1523972
doi: 10.1007/BF00311391
pmcid: 1523972
Vital C, Gherardi R, Vital A, Kopp N, Pellissier JF, Soubrier M, Clavelou P, Bellance R, Delisle MB, Ruchoux MM et al (1994) Uncompacted myelin lamellae in polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes syndrome. Ultrastructural study of peripheral nerve biopsy from 22 patients. Acta Neuropathol 87(3):302–7
pubmed: 8009962
doi: 10.1007/BF00296746
pmcid: 8009962
Ohnishi A, Hirano A (1981) Uncompacted myelin lamellae in dysglobulinemic neuropathy. J Neurol Sci 51(1):131–140
pubmed: 6265601
doi: 10.1016/0022-510X(81)90066-6
pmcid: 6265601
Hashimoto R, Koike H, Takahashi M, Ohyama K, Kawagashira Y, Iijima M, Sobue G (2015) Uncompacted myelin lamellae and nodal ion channel disruption in POEMS syndrome. J Neuropathol Exp Neurol 74(12):1127–1136
pubmed: 26574667
pmcid: 26574667
Yoshikawa H, Dyck PJ (1991) Uncompacted inner myelin lamellae in inherited tendency to pressure palsy. J Neuropathol Exp Neurol 50(5):649–657
pubmed: 1895146
doi: 10.1097/00005072-199109000-00009
pmcid: 1895146
Lagueny A, Latour P, Vital A, Le Masson G, Rouanet M, Ferrer X, Vital C, Vandenberghe A (2001) Mild recurrent neuropathy in CMT1B with a novel nonsense mutation in the extracellular domain of the MPZ gene. J Neurol Neurosurg Psychiatry 70(2):232–235
pubmed: 11160475
pmcid: 1737217
doi: 10.1136/jnnp.70.2.232
Fujinuma Y, Asahina M, Fukushima T, Katagiri A, Yamanaka Y, Misawa S, Kuwabara S (2012) Preserved autonomic function in patients with POEMS syndrome. J Neurol Sci 318(1–2):131–134
pubmed: 22507753
doi: 10.1016/j.jns.2012.03.004
pmcid: 22507753
Isose S, Misawa S, Kanai K, Shibuya K, Sekiguchi Y, Nasu S, Fujimaki Y, Noto Y, Nakaseko C, Kuwabara S (2011) POEMS syndrome with Guillan-Barre syndrome-like acute onset: a case report and review of neurological progression in 30 cases. J Neurol Neurosurg Psychiatry 82(6):678–680
pubmed: 20562460
doi: 10.1136/jnnp.2010.205369
pmcid: 20562460
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(5):476–479
pubmed: 22338030
doi: 10.1136/jnnp-2011-301706
pmcid: 22338030
Suichi T, Misawa S, Sato Y, Beppu M, Sakaida E, Sekiguchi Y, Shibuya K, Watanabe K, Amino H, Kuwabara S (2019) Proposal of new clinical diagnostic criteria for POEMS syndrome. J Neurol Neurosurg Psychiatry 90(2):133–137
pubmed: 30279213
doi: 10.1136/jnnp-2018-318514
pmcid: 30279213
Mauermann ML, Sorenson EJ, Dispenzieri A, Mandrekar J, Suarez GA, Dyck PJ, Dyck PJ (2012) Uniform demyelination and more severe axonal loss distinguish POEMS syndrome from CIDP. J Neurol Neurosurg Psychiatry 83(5):480–486
pubmed: 22396441
doi: 10.1136/jnnp-2011-301472
pmcid: 22396441
Marsh ES, Keddie S, Terris-Prestholt F, D’Sa S, Lunn MP (2021) Early VEGF testing in inflammatory neuropathy avoids POEMS syndrome misdiagnosis and associated costs. J Neurol Neurosurg Psychiatry 92(2):172–176
pubmed: 33106368
doi: 10.1136/jnnp-2020-324012
pmcid: 33106368
Sung JY, Kuwabara S, Ogawara K, Kanai K, Hattori T (2002) Patterns of nerve conduction abnormalities in POEMS syndrome. Muscle Nerve 26(2):189–193
pubmed: 12210381
doi: 10.1002/mus.10182
pmcid: 12210381
Sekiguchi Y, Misawa S, Shibuya K, Nasu S, Mitsuma S, Iwai Y, Beppu M, Sawai S, Ito S, Hirano S, Nakaseko C, Kuwabara S (2013) Ambiguous effects of anti-VEGF monoclonal antibody (bevacizumab) for POEMS syndrome. J Neurol Neurosurg Psychiatry 84(12):1346–1348
pubmed: 23463868
doi: 10.1136/jnnp-2012-304874
pmcid: 23463868
Huang CC, Chu CC (1996) Poor response to intravenous immunoglobulin therapy in patients with Castleman’s disease and the POEMS syndrome. J Neurol 243(10):726–727
pubmed: 8923307
doi: 10.1007/BF00873980
pmcid: 8923307
Brown R, Ginsberg L (2019) POEMS syndrome: clinical update. J Neurol 266(1):268–277
pubmed: 30498913
doi: 10.1007/s00415-018-9110-6
pmcid: 30498913
Cerri F, Falzone YM, Riva N, Quattrini A (2019) An update on the diagnosis and management of the polyneuropathy of POEMS syndrome. J Neurol 266(1):258–267
pubmed: 30264176
doi: 10.1007/s00415-018-9068-4
pmcid: 30264176
Humeniuk MS, Gertz MA, Lacy MQ, Kyle RA, Witzig TE, Kumar SK, Kapoor P, Lust JA, Hayman SR, Buadi FK, Rajkumar SV, Zeldenrust SR, Russell SJ, Dingli D, Lin Y, Leung N, Dispenzieri A (2013) Outcomes of patients with POEMS syndrome treated initially with radiation. Blood 122(1):68–73
pubmed: 23699599
pmcid: 4067496
doi: 10.1182/blood-2013-03-487025
Kuwabara S, Hattori T, Shimoe Y, Kamitsukasa I (1997) Long term melphalan-prednisolone chemotherapy for POEMS syndrome. J Neurol Neurosurg Psychiatry 63(3):385–387
pubmed: 9328260
pmcid: 2169695
doi: 10.1136/jnnp.63.3.385
Li J, Zhang W, Jiao L, Duan MH, Guan HZ, Zhu WG, Tian Z, Zhou DB (2011) Combination of melphalan and dexamethasone for patients with newly diagnosed POEMS syndrome. Blood 117(24):6445–6449
pubmed: 21393478
pmcid: 3123016
doi: 10.1182/blood-2010-12-328112
Ohwada C, Sakaida E, Kawajiri-Manako C, Nagao Y, Oshima-Hasegawa N, Togasaki E, Muto T, Tsukamoto S, Mitsukawa S, Takeda Y, Mimura N, Takeuchi M, Shimizu N, Misawa S, Iseki T, Kuwabara S, Nakaseko C (2018) Long-term evaluation of physical improvement and survival of autologous stem cell transplantation in POEMS syndrome. Blood 131(19):2173–2176
pubmed: 29599110
pmcid: 5946762
doi: 10.1182/blood-2017-07-795385
Misawa S, Sato Y, Katayama K, Nagashima K, Aoyagi R, Sekiguchi Y, Sobue G, Koike H, Yabe I, Sasaki H, Watanabe O, Takashima H, Nishizawa M, Kawachi I, Kusunoki S, Mitsui Y, Kikuchi S, Nakashima I, Ikeda S, Kohara N, Kanda T, Kira J, Hanaoka H, Kuwabara S, Japanese POEMS Syndrome for Thalidomide (J-POST) Trial Study Group (2016) Safety and efficacy of thalidomide in patients with POEMS syndrome: a multicentre, randomised, double-blind, placebo-controlled trial. Lancet Neurol 15(11):1129–37
pubmed: 27496680
doi: 10.1016/S1474-4422(16)30157-0
pmcid: 27496680
Delforge M, Bladé J, Dimopoulos MA, Facon T, Kropff M, Ludwig H, Palumbo A, Van Damme P, San-Miguel JF, Sonneveld P (2010) Treatment-related peripheral neuropathy in multiple myeloma: the challenge continues. Lancet Oncol 11(11):1086–1095
pubmed: 20932799
doi: 10.1016/S1470-2045(10)70068-1
pmcid: 20932799
Suichi T, Misawa S, Nagashima K, Sato Y, Iwai Y, Katayama K, Sekiguchi Y, Shibuya K, Amino H, Suzuki YI, Tsuneyama A, Nakamura K, Kuwabara S (2020) Lenalidomide treatment for thalidomide-refractory POEMS syndrome: a prospective single-arm clinical trial. Intern Med 59(9):1149–1153
pubmed: 32009091
pmcid: 7270764
doi: 10.2169/internalmedicine.3800-19
Gertz MA, Comenzo R, Falk RH, Fermand JP, Hazenberg BP, Hawkins PN, Merlini G, Moreau P, Ronco P, Sanchorawala V, Sezer O, Solomon A, Grateau G (2005) Definition of organ involvement and treatment response in immunoglobulin light chain amyloidosis (AL): a consensus opinion from the 10th International Symposium on Amyloid and Amyloidosis, Tours, France, 18–22 April 2004. Am J Hematol 79(4):319–328
pubmed: 16044444
doi: 10.1002/ajh.20381
pmcid: 16044444
Adams D (2001) Hereditary and acquired amyloid neuropathies. J Neurol 248(8):647–657
pubmed: 11569892
doi: 10.1007/s004150170109
pmcid: 11569892
Adams D, Koike H, Slama M, Coelho T (2019) Hereditary transthyretin amyloidosis: a model of medical progress for a fatal disease. Nat Rev Neurol 15(7):387–404
pubmed: 31209302
doi: 10.1038/s41582-019-0210-4
pmcid: 31209302
Vital C, Vallat JM, Deminiere C, Loubet A, Leboutet MJ (1982) Peripheral nerve damage during multiple myeloma and Waldenstrom’s macroglobulinemia: an ultrastructural and immunopathologic study. Cancer 50(8):1491–1497
pubmed: 6288215
doi: 10.1002/1097-0142(19821015)50:8<1491::AID-CNCR2820500808>3.0.CO;2-Q
pmcid: 6288215
Sommer C, Schröder JM (1989) Amyloid neuropathy: immunocytochemical localization of intra- and extracellular immunoglobulin light chains. Acta Neuropathol 79(2):190–199
pubmed: 2512758
doi: 10.1007/BF00294378
pmcid: 2512758
Koike H, Katsuno M (2019) Ultrastructure in transthyretin amyloidosis: from pathophysiology to therapeutic insights. Biomedicines 7(1):11
pmcid: 6466231
doi: 10.3390/biomedicines7010011
Mathis S, Magy L, Diallo L, Boukhris S, Vallat JM (2012) Amyloid neuropathy mimicking chronic inflammatory demyelinating polyneuropathy. Muscle Nerve 45(1):26–31
pubmed: 22190302
doi: 10.1002/mus.22229
pmcid: 22190302
Hamidi Asl K, Liepnieks JJ, Nakamura M, Benson MD (1999) Organ-specific (localized) synthesis of Ig light chain amyloid. J Immunol 162(9):5556–5560
pubmed: 10228037
pmcid: 10228037
Kyle RA, Greipp PR (1983) Amyloidosis (AL). Clinical and laboratory features in 229 cases. Mayo Clin Proc 58(10):665–83
pubmed: 6353084
pmcid: 6353084
Shimazaki C, Hata H, Iida S, Ueda M, Katoh N, Sekijima Y, Ikeda S, Yazaki M, Fukushima W, Ando Y (2018) Nationwide survey of 741 patients with systemic amyloid light-chain amyloidosis in Japan. Intern Med 57(2):181–187
pubmed: 29093404
doi: 10.2169/internalmedicine.9206-17
pmcid: 29093404
Koike H, Misu K, Sugiura M, Iijima M, Mori K, Yamamoto M, Hattori N, Mukai E, Ando Y, Ikeda S, Sobue G (2004) Pathology of early- vs late-onset TTR Met30 familial amyloid polyneuropathy. Neurology 63(1):129–138
pubmed: 15249622
doi: 10.1212/01.WNL.0000132966.36437.12
pmcid: 15249622
Koike H, Katsuno M (2020) Transthyretin amyloidosis: update on the clinical spectrum, pathogenesis, and disease-modifying therapies. Neurol Ther 9(2):317–333
pubmed: 32948978
pmcid: 7500251
doi: 10.1007/s40120-020-00210-7
Koike H, Tanaka F, Hashimoto R, Tomita M, Kawagashira Y, Iijima M, Fujitake J, Kawanami T, Kato T, Yamamoto M, Sobue G (2012) Natural history of transthyretin Val30Met familial amyloid polyneuropathy: analysis of late-onset cases from non-endemic areas. J Neurol Neurosurg Psychiatry 83(2):152–158
pubmed: 22228785
doi: 10.1136/jnnp-2011-301299
pmcid: 22228785
Gertz MA (2020) Immunoglobulin light chain amyloidosis: 2020 update on diagnosis, prognosis, and treatment. Am J Hematol 95(7):848–860
pubmed: 32267020
doi: 10.1002/ajh.25819
pmcid: 32267020
Koike H, Okumura T, Murohara T, Katsuno, M. Multidisciplinary approaches for transthyretin amyloidosis. Cardiol Ther., in press.
Klein CJ, Vrana JA, Theis JD, Dyck PJ, Dyck PJ, Spinner RJ, Mauermann ML, Bergen HR 3rd, Zeldenrust SR, Dogan A (2011) Mass spectrometric-based proteomic analysis of amyloid neuropathy type in nerve tissue. Arch Neurol 68(2):195–199
pubmed: 20937937
doi: 10.1001/archneurol.2010.261
pmcid: 20937937
Matsuda M, Gono T, Morita H, Katoh N, Kodaira M, Ikeda S (2011) Peripheral nerve involvement in primary systemic AL amyloidosis: a clinical and electrophysiological study. Eur J Neurol 18(4):604–610
pubmed: 20860753
doi: 10.1111/j.1468-1331.2010.03215.x
pmcid: 20860753
Jones NF, Hilton PJ, Tighe JR, Hobbs JR (1972) Treatment of “primary” renal amyloidosis with melphalan. Lancet 2(7778):616–619
pubmed: 4116774
doi: 10.1016/S0140-6736(72)93014-0
pmcid: 4116774
Kyle RA, Gertz MA, Greipp PR, Witzig TE, Lust JA, Lacy MQ, Therneau TM (1997) A trial of three regimens for primary amyloidosis: colchicine alone, melphalan and prednisone, and melphalan, prednisone, and colchicine. N Engl J Med 336(17):1202–1207
pubmed: 9110907
doi: 10.1056/NEJM199704243361702
pmcid: 9110907
Comenzo RL, Vosburgh E, Simms RW, Bergethon P, Sarnacki D, Finn K, Dubrey S, Faller DV, Wright DG, Falk RH, Skinner M (1996) Dose-intensive melphalan with blood stem cell support for the treatment of AL amyloidosis: one-year follow-up in five patients. Blood 88(7):2801–2806
pubmed: 8839879
doi: 10.1182/blood.V88.7.2801.bloodjournal8872801
pmcid: 8839879
Jaccard A, Moreau P, Leblond V, Leleu X, Benboubker L, Hermine O, Recher C, Asli B, Lioure B, Royer B, Jardin F, Bridoux F, Grosbois B, Jaubert J, Piette JC, Ronco P, Quet F, Cogne M, Fermand JP (2007) Myélome Autogreffe (MAG) and Intergroupe Francophone du Myélome (IFM) Intergroup. High-dose melphalan versus melphalan plus dexamethasone for AL amyloidosis. N Engl J Med 357(11):1083 93
pubmed: 17855669
doi: 10.1056/NEJMoa070484
pmcid: 17855669
Gertz MA, Lacy MQ, Dispenzieri A, Buadi FK, Dingli D, Hayman SR, Kumar SK, Leung N, Lust J, Rajkumar SV, Russell SJ, Suman VJ, Le-Rademacher JG, Hogan WJ (2016) Stem cell transplantation compared with melphalan plus dexamethasone in the treatment of immunoglobulin light-chain amyloidosis. Cancer 122(14):2197–2205
pubmed: 27142462
doi: 10.1002/cncr.30051
pmcid: 27142462
Obici L, Adams D (2020) Acquired and inherited amyloidosis: knowledge driving patients’ care. J Peripher Nerv Syst 25(2):85–101
pubmed: 32378274
doi: 10.1111/jns.12381
pmcid: 32378274
Biolo A, Ramamurthy S, Connors LH, O’Hara CJ, Meier-Ewert HK, Soo Hoo PT, Sawyer DB, Seldin DC, Sam F (2008) Matrix metalloproteinases and their tissue inhibitors in cardiac amyloidosis: relationship to structural, functional myocardial changes and to light chain amyloid deposition. Circ Heart Fail 1(4):249–257
pubmed: 19808299
pmcid: 2774449
doi: 10.1161/CIRCHEARTFAILURE.108.788687
Ward JE, Ren R, Toraldo G, Soohoo P, Guan J, O’Hara C, Jasuja R, Trinkaus-Randall V, Liao R, Connors LH, Seldin DC (2011) Doxycycline reduces fibril formation in a transgenic mouse model of AL amyloidosis. Blood 118(25):6610–6617
pubmed: 21998211
pmcid: 3242721
doi: 10.1182/blood-2011-04-351643
D’Souza A, Flynn K, Chhabra S, Dhakal B, Hamadani M, Jacobsen K, Pasquini M, Weihrauch D, Hari P (2017) Rationale and design of DUAL study: doxycycline to upgrade response in light chain (AL) amyloidosis (DUAL): a phase 2 pilot study of a two-pronged approach of prolonged doxycycline with plasma cell-directed therapy in the treatment of AL amyloidosis. Contemp Clin Trials Commun 24(8):33–38
doi: 10.1016/j.conctc.2017.08.012
Griffin JM, Rosenblum H, Maurer MS (2021) Pathophysiology and therapeutic approaches to cardiac amyloidosis. Circ Res 128(10):1554–1575
pubmed: 33983835
doi: 10.1161/CIRCRESAHA.121.318187
pmcid: 33983835
Hrncic R, Wall J, Wolfenbarger DA, Murphy CL, Schell M, Weiss DT, Solomon A (2000) Antibody-mediated resolution of light chain-associated amyloid deposits. Am J Pathol 157(4):1239–1246
pubmed: 11021828
pmcid: 1850152
doi: 10.1016/S0002-9440(10)64639-1
Nardelli E, Steck AJ, Barkas T, Schluep M, Jerusalem F (1988) Motor neuron syndrome and monoclonal IgM with antibody activity against gangliosides GM1 and GD1b. Ann Neurol 23(5):524–528
pubmed: 3389760
doi: 10.1002/ana.410230517
pmcid: 3389760
Eurelings M, Ang CW, Notermans NC, Van Doorn PA, Jacobs BC, Van den Berg LH (2001) Antiganglioside antibodies in polyneuropathy associated with monoclonal gammopathy. Neurology 57(10):1909–1912
pubmed: 11723289
doi: 10.1212/WNL.57.10.1909
pmcid: 11723289
Vlam L, Piepers S, Sutedja NA, Jacobs BC, Tio-Gillen AP, Stam M, Franssen H, Veldink JH, Cats EA, Notermans NC, Bloem AC, Wadman RI, van der Pol WL, van den Berg LH (2015) Association of IgM monoclonal gammopathy with progressive muscular atrophy and multifocal motor neuropathy: a case-control study. J Neurol 262(3):666–673
pubmed: 25549972
doi: 10.1007/s00415-014-7612-4
pmcid: 25549972
Carpo M, Meucci N, Allaria S, Marmiroli P, Monaco S, Toscano A, Simonetti S, Scarlato G, Nobile-Orazio E (2000) Anti-sulfatide IgM antibodies in peripheral neuropathy. J Neurol Sci 176(2):144–150
pubmed: 10930598
doi: 10.1016/S0022-510X(00)00342-7
pmcid: 10930598
Dabby R, Weimer LH, Hays AP, Olarte M, Latov N (2000) Antisulfatide antibodies in neuropathy: clinical and electrophysiologic correlates. Neurology 54(7):1448–1452
pubmed: 10751255
doi: 10.1212/WNL.54.7.1448
pmcid: 10751255
Matà S, Borsini W, Ambrosini S, Toscani L, Barilaro A, Piacentini S, Sorbi S, Lolli F (2011) IgM monoclonal gammopathy-associated neuropathies with different IgM specificity. Eur J Neurol 18(8):1067–1073
pubmed: 21261794
doi: 10.1111/j.1468-1331.2010.03345.x
pmcid: 21261794
Campagnolo M, Ferrari S, Dalla Torre C, Cabrini I, Cacciavillani M, Lucchetta M, Ruggero S, Toffanin E, Cavallaro T, Briani C (2015) Polyneuropathy with anti-sulfatide and anti-MAG antibodies: clinical, neurophysiological, pathological features and response to treatment. J Neuroimmunol 15(281):1–4
doi: 10.1016/j.jneuroim.2015.02.009
Boso F, Ruggero S, Giannotta C, Benedetti L, Marfia GA, Ermani M, Campagnolo M, Salvalaggio A, Gallia F, De Michelis C, Visentin A, Bianco M, Ruiz M, Mataluni G, Nobile-Orazio E, Briani C (2017) Anti-sulfatide/galactocerebroside antibodies in immunoglobulin M paraproteinemic neuropathies. Eur J Neurol 24(11):1334–1340
pubmed: 28782863
doi: 10.1111/ene.13387
pmcid: 28782863
Willison HJ, O’Leary CP, Veitch J, Blumhardt LD, Busby M, Donaghy M, Fuhr P, Ford H, Hahn A, Renaud S, Katifi HA, Ponsford S, Reuber M, Steck A, Sutton I, Schady W, Thomas PK, Thompson AJ, Vallat JM, Winer J (2001) The clinical and laboratory features of chronic sensory ataxic neuropathy with anti-disialosyl IgM antibodies. Brain 124(Pt 10):1968–1977
pubmed: 11571215
doi: 10.1093/brain/124.10.1968
pmcid: 11571215