Abstract
Plasma cell disorders include monoclonal gammopathies of unknown significance, multiple myeloma, plasmacytoma, Waldenstrom’s macroglobulinemia, and amyloidosis. This group of neoplastic diseases involves proliferation of a single clone of plasma cells, producing a monoclonal immunoglobulin protein or immunoglobulin fragment (M protein) that can be identified as a spike on electrophoresis of serum or urine. Plasma cell disorders are associated with a wide spectrum of neurological complications. The pathophysiology, diagnosis, and management of these neurological complications are the subject of this review.
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References
Dispenzieri A, Kyle RA (2005) Neurological aspects of multiple myeloma and related disorders. Best Pract Res Clin Haematol 18:673–688
Spinazze S, Caraceni A, Schrijvers D (2005) Epidural spinal cord compression. Crit Rev Oncol Hematol 56:397–406
Maghfoor I, Perry MC (2000) Malignancy: meningeal myeloma: a case report and review of literature. Hematology 5:47–52
Roddie P, Collie D, Johnson P (2000) Myelomatous involvement of the dura mater: a rare complication of multiple myeloma. J Clin Pathol 53:398–399
Savage DG, Mears JG, Balmaceda C et al (2002) Leptomeningeal relapse of multiple myeloma following allogeneic stem cell transplantation. Leuk Res 26:689–692
Slager UT, Taylor WF, Opfell RW, Myers A (1979) Leptomeningeal myeloma. Arch Pathol Lab Med 103:680–682
Oyajobi BO (2007) Multiple myeloma/hypercalcemia. Arthritis Res Ther 9(Suppl 1):S4
Mehta J, Singhal S (2003) Hyperviscosity syndrome in plasma cell dyscrasias. Semin Thromb Hemost 29:467–471
Johnson SA, Oscier DG, Leblond V (2002) Waldenstrom’s macroglobulinaemia. Blood Rev 16:175–184
Preston FE, Cooke KB, Foster ME et al (1978) Myelomatosis and the hyperviscosity syndrome. Br J Haematol 38:517–530
Pruzanski W, Watt JG (1972) Serum viscosity and hyperviscosity syndrome in IgG multiple myeloma. Report on 10 patients and a review of the literature. Ann Intern Med 77:853–860
Adams BD, Baker R, Lopez JA, Spencer S (2009) Myeloproliferative disorders and the hyperviscosity syndrome. Emerg Med Clin North Am 27:459–476
Pavy MD, Murphy PL, Virella G (1980) Paraprotein-induced hyperviscosity. A reversible cause of stroke. Postgrad Med 68:109–112
Stone MJ (2009) Waldenstrom’s macroglobulinemia: hyperviscosity syndrome and cryoglobulinemia. Clin Lymphoma Myeloma 9:97–99
Bernard A, Rousselie F (1986) Macular manifestations of monoclonal dysgammaglobulinemias. Apropos of 3 cases. J Fr Ophthalmol 9:805–810
Alatoom A, Elsabrouty R, Willis J et al (2009) Fatal cerebral hemorrhage in a patient with CD19-positive IgM-producing aggressive plasma cell myeloma, hyperviscosity syndrome and cryoglobulinemia. Int J Clin Exp Pathol 2:498–507
Chen C, Kouroukis CT, White D et al (2009) Bortezomib in relapsed or refractory Waldenstrom’s macroglobulinemia. Clin Lymphoma Myeloma 9:74–76
Dimopoulos MA, Gertz MA, Kastritis E et al (2009) Update on treatment recommendations from the Fourth International Workshop on Waldenstrom’s Macroglobulinemia. J Clin Oncol 27:120–126
Treon SP (2009) How I treat Waldenstrom macroglobulinemia. Blood 114:2375–2385
Bing JN, Fog M, Neel AV (1937) Report of a third case of hyperglobulinemia with affection of the central nervous system on a toxi-infectious basis, and some remarks on the differential diagnosis. Acta Med. Scand 91:409–427
Fintelmann F, Forghani R, Schaefer PW et al (2009) Bing-Neel syndrome revisited. Clin Lymphoma Myeloma 9:104–106
Edgar R, Dutcher TF (1961) Histopathology of the Bing-Neel syndrome. Neurology 11:239–245
Grewal JS, Brar PK, Sahijdak WM et al (2009) Bing-Neel syndrome: a case report and systematic review of clinical manifestations, diagnosis, and treatment options. Clin Lymphoma Myeloma 9:462–466
Malkani RG, Tallman M, Gottardi-Littell N et al (2009) Bing-Neel syndrome: an illustrative case and a comprehensive review of the published literature. J Neurooncol 96(3):301–312
Welch D, Whetsell WO Jr., Weil RJ (2002) Pathologic quiz case. A man with long-standing monoclonal gammopathy and new onset of confusion. Central nervous system involvement by Waldenstrom macroglobulinemia-Bing-Neel syndrome. Arch Pathol Lab Med 126:1243–1244
Drappatz J, Akar S, Fisher DC et al (2008) Imaging of Bing-Neel syndrome. Neurology 70:1364
Fassas AB, Ward S, Muwalla F et al (2004) Myeloma of the central nervous system: strong association with unfavorable chromosomal abnormalities and other high-risk disease features. Leuk Lymphoma 45:291–300
Nieuwenhuizen L, Biesma DH (2008) Central nervous system myelomatosis: review of the literature. Eur J Haematol 80:1–9
Dennis M, Chu P (2000) A case of meningeal myeloma presenting as obstructive hydrocephalus – a therapeutic challenge. Leuk Lymphoma 40:219–220
Pontikoglou C, Fragos C, Kolyvaki E et al (2005) Multiple myeloma involving the central nervous system: a report of two cases with unusual manifestations. Leuk Lymphoma 46:737–741
Quach H, Ryan G, Ganju V, Prince HM (2005) Effective treatment of leptomeningeal multiple myeloma with total craniospinal irradiation supported by second allogeneic donor stem cell infusion. Bone Marrow Transplant 35:423–424
Veinstein A, Brizard A, Randriamalala E et al (1997) Central nervous system relapses after autologous stem cell transplantation for myeloma. Report of two cases. Hematol Cell Ther 39:327–330
Hogan MC, Lee A, Solberg LA, Thome SD (2002) Unusual presentation of multiple myeloma with unilateral visual loss and numb chin syndrome in a young adult. Am J Hematol 70:55–59
Knapp AJ, Gartner S, Henkind P (1987) Multiple myeloma and its ocular manifestations. Surv Ophthalmol 31:343–351
Shone GR (1985) Facial palsy due to myeloma of the temporal bone. J Laryngol Otol 99:907–908
Witt C, Borges AC, Klein K, Neumann HJ (1997) Radiographic manifestations of multiple myeloma in the mandible: a retrospective study of 77 patients. J Oral Maxillofac Surg 55:450–453; discussion 454–455
Colak A, Cataltepe O, Ozgen T, Erbengi A (1989) Spinal cord compression caused by plasmacytomas. A retrospective review of 14 cases. Neurosurg Rev 12:305–308
Spiess JL, Adelstein DJ, Hines JD (1988) Multiple myeloma presenting with spinal cord compression. Oncology 45:88–92
Schiff D (2003) Spinal cord compression. Neurol Clin 21:67–86
Young RF, Post EM, King GA (1980) Treatment of spinal epidural metastases. Randomized prospective comparison of laminectomy and radiotherapy. J Neurosurg 53:741–748
Patchell RA, Tibbs PA, Regine WF et al (2005) Direct decompressive surgical resection in the treatment of spinal cord compression caused by metastatic cancer: a randomised trial. Lancet 366:643–648
Witham TF, Khavkin YA, Gallia GL et al (2006) Surgery insight: current management of epidural spinal cord compression from metastatic spine disease. Nat Clin Pract Neurol 2:87–94; quiz 116
Sun H, Nemecek AN (2009) Optimal management of malignant epidural spinal cord compression. Emerg Med Clin North Am 27:195–208
Costa L, Major PP (2009) Effect of bisphosphonates on pain and quality of life in patients with bone metastases. Nat Clin Pract Oncol 6:163–174
Kyle RA, Gertz MA, Witzig TE et al (2003) Review of 1027 patients with newly diagnosed multiple myeloma. Mayo Clin Proc 78:21–33
Mundy GR (1998) Myeloma bone disease. Eur J Cancer 34:246–251
Riggs JE (2002) Neurologic manifestations of electrolyte disturbances. Neurol Clin 20:227–239
Nobile-Orazio E, Barbieri S, Baldini L et al (1992) Peripheral neuropathy in monoclonal gammopathy of undetermined significance: prevalence and immunopathogenetic studies. Acta Neurol Scand 85:383–390
Vrethem M, Cruz M, Wen-Xin H et al (1993) Clinical, neurophysiological and immunological evidence of polyneuropathy in patients with monoclonal gammopathies. J Neurol Sci 114:193–199
Silverstein A, Doniger DE (1963) Neurologic complications of myelomatosis. Arch Neurol 9:534–544
Walsh JC (1971) The neuropathy of multiple myeloma. An electrophysiological and histological study. Arch Neurol 25:404–414
Richardson PG, Xie W, Mitsiades C et al (2009) Single-agent bortezomib in previously untreated multiple myeloma: efficacy, characterization of peripheral neuropathy, and molecular correlations with response and neuropathy. J Clin Oncol 27:3518–3525
Kelly JJ Jr., Kyle RA, O‘Brien PC, Dyck PJ (1981) Prevalence of monoclonal protein in peripheral neuropathy. Neurology 31:1480–1483
Ramchandren S, Lewis RA (2009) Monoclonal gammopathy and neuropathy. Curr Opin Neurol 22:480–485
Latov N, Braun PE, Gross RB et al (1981) Plasma cell dyscrasia and peripheral neuropathy: identification of the myelin antigens that react with human paraproteins. Proc Natl Acad Sci USA 78:7139–7142
Latov N, Hays AP, Sherman WH (1988) Peripheral neuropathy and anti-MAG antibodies. Crit Rev Neurobiol 3:301–332
Ellie E, Vital A, Steck A et al (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 243:34–43
Yeung KB, Thomas PK, King RH et al (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 238:383–391
Drappatz J, Batchelor T (2004) Neurologic complications of plasma cell disorders. Clin Lymphoma 5:163–171
Eurelings M, Moons KG, Notermans NC et al (2001) Neuropathy and IgM M-proteins: prognostic value of antibodies to MAG, SGPG, and sulfatide. Neurology 56:228–233
Nobile-Orazio E, Carpo M (2001) Neuropathy and monoclonal gammopathy. Curr Opin Neurol 14:615–620
Zivkovic SA, Lacomis D, Lentzsch S (2009) Paraproteinemic neuropathy. Leuk Lymphoma 50:1422–1433
Lunn MP, Nobile-Orazio E (2003) Immunotherapy for IgM anti-Myelin-associated glycoprotein paraprotein-associated peripheral neuropathies. Cochrane Database Syst Rev 1:CD002827
Pestronk A, Florence J, Miller T et al (2003) Treatment of IgM antibody associated polyneuropathies using rituximab. J Neurol Neurosurg Psychiatry 74:485–489
Dalakas MC, Rakocevic G, Salajegheh M et al (2009) Placebo-controlled trial of rituximab in IgM anti-myelin-associated glycoprotein antibody demyelinating neuropathy. Ann Neurol 65:286–293
Vrethem M, Reiser N, Lauermann C, Svanborg E (2009) Polyneuropathy associated with IgM vs IgG monoclonal gammopathy: comparison between clinical and electrophysiological findings. Acta Neurol Scand 122(1):52–57
Gorson KC, Ropper AH, Weinberg DH, Weinstein R (2002) Efficacy of intravenous immunoglobulin in patients with IgG monoclonal gammopathy and polyneuropathy. Arch Neurol 59:766–772
Wicklund MP, Kissel JT (2001) Paraproteinemic neuropathy. Curr Treat Options Neurol 3:147–156
Hadden RD, Nobile-Orazio E, Sommer C et al (2006) European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of paraproteinaemic demyelinating neuropathies: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society. Eur J Neurol 13:809–818
Victor M, Banker BQ, Adams RD (1958) The neuropathy of multiple myeloma. J Neurol Neurosurg Psychiatry 21:73–88
Briemberg HR, Amato AA (2003) Neuromuscular complications of cancer. Neurol Clin 21:141–165
Nakanishi T, Sobue I, Toyokura Y et al (1984) The Crow-Fukase syndrome: a study of 102 cases in Japan. Neurology 34:712–720
Kelly JJ Jr., Kyle RA, Miles JM, Dyck PJ (1983) Osteosclerotic myeloma and peripheral neuropathy. Neurology 33:202–210
Kuwabara S, Misawa S, Kanai K et al (2008) Thalidomide reduces serum VEGF levels and improves peripheral neuropathy in POEMS syndrome. J Neurol Neurosurg Psychiatry 79:1255–1257
Steck AJ, Czaplinski A, Renaud S (2008) Inflammatory demyelinating neuropathies and neuropathies associated with monoclonal gammopathies: treatment update. Neurotherapeutics 5:528–534
Creange A, Chater A, Brouet JC et al (2008) A case of POEMS syndrome treated by autologous hematopoietic stem-cell transplantation. Nat Clin Pract Neurol 4:686–691
Gupta S, Rana V, Chandra D et al (2006) Autologous peripheral stem cell transplant for POEMS syndrome: a case report. Hematology 11:361–363
Mahdi-Rogers M, Kazmi M, Ferner R et al (2009) Autologous peripheral blood stem cell transplantation for chronic acquired demyelinating neuropathy. J Peripher Nerv Syst 14:118–124
Plasmati R, Pastorelli F, Cavo M et al (2007) Neuropathy in multiple myeloma treated with thalidomide: a prospective study. Neurology 69:573–581
Richardson PG, Briemberg H, Jagannath S et al (2006) Frequency, characteristics, and reversibility of peripheral neuropathy during treatment of advanced multiple myeloma with bortezomib. J Clin Oncol 24:3113–3120
Julien J, Vital C, Vallat JM et al (1978) Polyneuropathy in Waldenstrom’s macroglobulinemia. Deposition of M component on myelin sheaths. Arch Neurol 35:423–425
Rudnicki SA, Harik SI, Dhodapkar M et al (1998) Nervous system dysfunction in Waldenstrom’s macroglobulinemia: response to treatment. Neurology 51:1210–1213
Vital C, Deminiere C, Bourgouin B et al (1985) Waldenstrom’s macroglobulinemia and peripheral neuropathy: deposition of M-component and kappa light chain in the endoneurium. Neurology 35:603–606
Adams D (2001) Hereditary and acquired amyloid neuropathies. J Neurol 248:647–657
Kyle RA, Greipp PR (1983) Amyloidosis (AL). Clinical and laboratory features in 229 cases. Mayo Clin Proc 58:665–683
Bloch KJ, Franklin E (1982) Plasma cell dyscrasias and cryoglobulins. JAMA 248:2670–2676
Garcia-Bragado F, Fernandez JM, Navarro C et al (1988) Peripheral neuropathy in essential mixed cryoglobulinemia. Arch Neurol 45:1210–1214
Nemni R, Corbo M, Fazio R et al (1988) Cryoglobulinaemic neuropathy. A clinical, morphological and immunocytochemical study of 8 cases. Brain 111(Pt 3):541–552
Vernino S, Wolfe GI (2007) Antibody testing in peripheral neuropathies. Neurol Clin 25:29–46
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Drappatz, J., Jaeckle, K.A. (2012). Neurological Complications of Plasma Cell Disorders. In: Batchelor, T., DeAngelis, L. (eds) Lymphoma and Leukemia of the Nervous System. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-7668-0_18
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