Skip to main content
Erschienen in: Journal of Neuro-Oncology 3/2010

01.02.2010 | Topic Review

Bing–Neel syndrome: an illustrative case and a comprehensive review of the published literature

verfasst von: Roneil G. Malkani, Martin Tallman, Numa Gottardi-Littell, William Karpus, Laura Marszalek, Daina Variakojis, Bruce Kaden, Matthew Walker, Robert M. Levy, Jeffrey J. Raizer

Erschienen in: Journal of Neuro-Oncology | Ausgabe 3/2010

Einloggen, um Zugang zu erhalten

Abstract

Waldenstrom’s macroglobulinemia (WM) is a chronic lymphoproliferative disorder within the spectrum of lymphoplasmacytic lymphoma characterized by proliferation of plasma cells, small lymphocytes, and plasmacytoid lymphocytes. Central nervous system involvement is very rare (Bing–Neel [BN] syndrome). We present the case of a 62-year-old woman previously diagnosed with WM who presented with Bing–Neel syndrome and review the published literature which consists of only case reports. We performed a Medline search using the terms “Waldenstrom’s macroglobulinemia and central nervous system” and “Bing–Neel” collecting data on presentation, evaluation, treatment, and outcome and summarizing these findings in the largest pooled series to date. Central nervous system manifestations are localization related. Serum laboratory testing reflects systemic disease. Cerebrospinal fluid analysis may show lymphocytic pleocytosis, elevated protein, and IgM kappa or lambda light chain restriction; cytology results are variable. Imaging is frequently abnormal. Biopsy confirms the diagnosis. Treatment data are limited, but responses are seen with radiation and/or chemotherapy. BN syndrome is a very rare complication of WM that should be considered in patients with neurologic symptoms and a history of WM. Treatment should be initiated as responses do occur that may improve quality of life and extend it when limited or no active systemic disease is present.
Literatur
1.
Zurück zum Zitat Swerdlow SH, International Agency for Research on Cancer, World Health Organization (2008) WHO classification of tumours of haematopoietic and lymphoid tissues. International Agency for Research on Cancer, Lyon, France Swerdlow SH, International Agency for Research on Cancer, World Health Organization (2008) WHO classification of tumours of haematopoietic and lymphoid tissues. International Agency for Research on Cancer, Lyon, France
2.
Zurück zum Zitat Waldenstrom J (1944) Incipient myelomatosis or “essential” hyperglobulinemia with fibrogenopenia: a new syndrome? Acta Med Scand 117:216–247 Waldenstrom J (1944) Incipient myelomatosis or “essential” hyperglobulinemia with fibrogenopenia: a new syndrome? Acta Med Scand 117:216–247
3.
Zurück zum Zitat Bing J, Neel A (1936) Two cases of hyperglobulinemia with affection of the central nervous system on a toxi-infection bases. Acta Med Scand 88:492–506 Bing J, Neel A (1936) Two cases of hyperglobulinemia with affection of the central nervous system on a toxi-infection bases. Acta Med Scand 88:492–506
4.
Zurück zum Zitat Vula O, Merhaien P (1972) Cerebral reticuloplasmacytosis with hyperglobulinemia. Its relation to the Bing-Neel syndrome. Eur Neurol 7:155–168CrossRefPubMed Vula O, Merhaien P (1972) Cerebral reticuloplasmacytosis with hyperglobulinemia. Its relation to the Bing-Neel syndrome. Eur Neurol 7:155–168CrossRefPubMed
5.
Zurück zum Zitat Imai F, Fujisawa K, Kiya N, Ninomiya T, Ogura Y, Mizoguchi Y, Sano H, Kanno T (1995) Intracerebral infiltration by monoclonal plasmacytoid cells in Waldenstrom’s macroglobulinemia—case report. Neurol Med Chir (Tokyo) 35:575–579CrossRef Imai F, Fujisawa K, Kiya N, Ninomiya T, Ogura Y, Mizoguchi Y, Sano H, Kanno T (1995) Intracerebral infiltration by monoclonal plasmacytoid cells in Waldenstrom’s macroglobulinemia—case report. Neurol Med Chir (Tokyo) 35:575–579CrossRef
6.
Zurück zum Zitat Logothetis J, Silverstein P, Coe J (1960) Neurologic aspects of Waldenstrom’s macroglobulinemia; report of a case. Arch Neurol 3:564–573PubMed Logothetis J, Silverstein P, Coe J (1960) Neurologic aspects of Waldenstrom’s macroglobulinemia; report of a case. Arch Neurol 3:564–573PubMed
7.
Zurück zum Zitat Edgar R, Dutcher TF (1961) Histopathology of the Bing-Neel syndrome. Neurology 11:239–245PubMed Edgar R, Dutcher TF (1961) Histopathology of the Bing-Neel syndrome. Neurology 11:239–245PubMed
8.
Zurück zum Zitat Bhatti MT, Yuan C, Winter W, McSwain AS, Okun MS (2005) Bilateral sixth nerve paresis in the Bing-Neel syndrome. Neurology 64:576–577PubMed Bhatti MT, Yuan C, Winter W, McSwain AS, Okun MS (2005) Bilateral sixth nerve paresis in the Bing-Neel syndrome. Neurology 64:576–577PubMed
9.
Zurück zum Zitat Torrey JJ, Katakkar SB (1984) Treatable meningeal involvement in Waldenstrom’s macroglobulinemia. Ann Intern Med 101:345–347PubMed Torrey JJ, Katakkar SB (1984) Treatable meningeal involvement in Waldenstrom’s macroglobulinemia. Ann Intern Med 101:345–347PubMed
10.
Zurück zum Zitat Neau JP, Guilhot F, Dumas P, Besson I, Rivasseau-Jonveaux T, Gil R (1991) [Central neurologic forms of Waldenstrom’s disease Bing-Neel syndrome. 3 cases]. Rev Neurol (Paris) 147:56–60 Neau JP, Guilhot F, Dumas P, Besson I, Rivasseau-Jonveaux T, Gil R (1991) [Central neurologic forms of Waldenstrom’s disease Bing-Neel syndrome. 3 cases]. Rev Neurol (Paris) 147:56–60
11.
Zurück zum Zitat Delgado J, Canales MA, Garcia B, Alvarez-Ferreira J, Garcia-Grande A, Hernandez-Navarro F (2002) Radiation therapy and combination of cladribine, cyclophosphamide, and prednisone as treatment of Bing-Neel syndrome: case report and review of the literature. Am J Hematol 69:127–131CrossRefPubMed Delgado J, Canales MA, Garcia B, Alvarez-Ferreira J, Garcia-Grande A, Hernandez-Navarro F (2002) Radiation therapy and combination of cladribine, cyclophosphamide, and prednisone as treatment of Bing-Neel syndrome: case report and review of the literature. Am J Hematol 69:127–131CrossRefPubMed
12.
Zurück zum Zitat Donix M, Beuthien-Baumann B, von Kummer R, Gahn G, Thomas F, Holthoff V (2007) Nonfluent aphasia in a patient with Waldenstrom’s macroglobulinemia. J Clin Neurosci 14:601–603CrossRefPubMed Donix M, Beuthien-Baumann B, von Kummer R, Gahn G, Thomas F, Holthoff V (2007) Nonfluent aphasia in a patient with Waldenstrom’s macroglobulinemia. J Clin Neurosci 14:601–603CrossRefPubMed
13.
Zurück zum Zitat Fain O, Wechsler B, Vidailhet M, Raphael M, Schuller E, Godeau P (1992) [Involvement of central nervous system disclosing Waldenstrom’s disease: demonstration of intrathecal secretion of immunoglobulin M]. Rev Med Interne 13:58–60CrossRefPubMed Fain O, Wechsler B, Vidailhet M, Raphael M, Schuller E, Godeau P (1992) [Involvement of central nervous system disclosing Waldenstrom’s disease: demonstration of intrathecal secretion of immunoglobulin M]. Rev Med Interne 13:58–60CrossRefPubMed
14.
Zurück zum Zitat Garderet L, Baudel JL, Cervera P, Azizi L, Maury E, Guidet B, Gorin NC, Offenstadt G (2006) ‘Indolent’ Waldenstrom’s macroglobulinemia and a cerebrospinal fluid protein level of 16 g/L. Eur J Haematol 77:80–82CrossRefPubMed Garderet L, Baudel JL, Cervera P, Azizi L, Maury E, Guidet B, Gorin NC, Offenstadt G (2006) ‘Indolent’ Waldenstrom’s macroglobulinemia and a cerebrospinal fluid protein level of 16 g/L. Eur J Haematol 77:80–82CrossRefPubMed
15.
Zurück zum Zitat 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 126:1243–1244PubMed 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 126:1243–1244PubMed
16.
Zurück zum Zitat Scheithauer BW, Rubinstein LJ, Herman MM (1984) Leukoencephalopathy in Waldenstrom’s macroglobulinemia. Immunohistochemical and electron microscopic observations. J Neuropathol Exp Neurol 43:408–425CrossRefPubMed Scheithauer BW, Rubinstein LJ, Herman MM (1984) Leukoencephalopathy in Waldenstrom’s macroglobulinemia. Immunohistochemical and electron microscopic observations. J Neuropathol Exp Neurol 43:408–425CrossRefPubMed
17.
Zurück zum Zitat Weiss AH, Smith E, Christoff N, Kochwa S (1965) Cerebrospinal fluid paraproteins in multiple myeloma. J Lab Clin Med 66:280–293PubMed Weiss AH, Smith E, Christoff N, Kochwa S (1965) Cerebrospinal fluid paraproteins in multiple myeloma. J Lab Clin Med 66:280–293PubMed
18.
Zurück zum Zitat Frantzen E, Hertz H, Matzke J, Videbaek A (1969) Protein studies on cerebrospinal fluid and neurological symptoms in myelomatosis. Acta Neurol Scand 45:1–17CrossRefPubMed Frantzen E, Hertz H, Matzke J, Videbaek A (1969) Protein studies on cerebrospinal fluid and neurological symptoms in myelomatosis. Acta Neurol Scand 45:1–17CrossRefPubMed
19.
Zurück zum Zitat Hansotia P, Gani K, Friedenberg W (1983) Cerebrospinal fluid monoclonal gammopathy in multiple myeloma and Waldenstrom’s macroglobulinemia. Neurology 33:1411–1415PubMed Hansotia P, Gani K, Friedenberg W (1983) Cerebrospinal fluid monoclonal gammopathy in multiple myeloma and Waldenstrom’s macroglobulinemia. Neurology 33:1411–1415PubMed
20.
Zurück zum Zitat Dimopoulos MA, Kantarjian H, Weber D, O’Brien S, Estey E, Delasalle K, Rose E, Cabanillas F, Keating M, Alexanian R (1994) Primary therapy of Waldenstrom’s macroglobulinemia with 2-chlorodeoxyadenosine. J Clin Oncol 12:2694–2698PubMed Dimopoulos MA, Kantarjian H, Weber D, O’Brien S, Estey E, Delasalle K, Rose E, Cabanillas F, Keating M, Alexanian R (1994) Primary therapy of Waldenstrom’s macroglobulinemia with 2-chlorodeoxyadenosine. J Clin Oncol 12:2694–2698PubMed
21.
Zurück zum Zitat Laurencet FM, Zulian GB, Guetty-Alberto M, Iten PA, Betticher DC, Alberto P (1999) Cladribine with cyclophosphamide and prednisone in the management of low-grade lymphoproliferative malignancies. Br J Cancer 79:1215–1219CrossRefPubMed Laurencet FM, Zulian GB, Guetty-Alberto M, Iten PA, Betticher DC, Alberto P (1999) Cladribine with cyclophosphamide and prednisone in the management of low-grade lymphoproliferative malignancies. Br J Cancer 79:1215–1219CrossRefPubMed
22.
Zurück zum Zitat Richards AI (1995) Response of meningeal Waldenstrom’s macroglobulinemia to 2-chlorodeoxyadenosine. J Clin Oncol 13:2476PubMed Richards AI (1995) Response of meningeal Waldenstrom’s macroglobulinemia to 2-chlorodeoxyadenosine. J Clin Oncol 13:2476PubMed
23.
Zurück zum Zitat Dimopoulos MA, Anagnostopoulos A, Kyrtsonis MC, Zervas K, Tsatalas C, Kokkinis G, Repoussis P, Symeonidis A, Delimpasi S, Katodritou E, Vervessou E, Michali E, Pouli A, Gika D, Vassou A, Terpos E, Anagnostopoulos N, Economopoulos T, Pangalis G (2007) Primary treatment of Waldenstrom macroglobulinemia with dexamethasone, rituximab, and cyclophosphamide. J Clin Oncol 25:3344–3349CrossRefPubMed Dimopoulos MA, Anagnostopoulos A, Kyrtsonis MC, Zervas K, Tsatalas C, Kokkinis G, Repoussis P, Symeonidis A, Delimpasi S, Katodritou E, Vervessou E, Michali E, Pouli A, Gika D, Vassou A, Terpos E, Anagnostopoulos N, Economopoulos T, Pangalis G (2007) Primary treatment of Waldenstrom macroglobulinemia with dexamethasone, rituximab, and cyclophosphamide. J Clin Oncol 25:3344–3349CrossRefPubMed
24.
Zurück zum Zitat Treon SP, Emmanouilides C, Kimby E, Kelliher A, Preffer F, Branagan AR, Anderson KC, Frankel SR (2005) Extended rituximab therapy in Waldenstrom’s macroglobulinemia. Ann Oncol 16:132–138CrossRefPubMed Treon SP, Emmanouilides C, Kimby E, Kelliher A, Preffer F, Branagan AR, Anderson KC, Frankel SR (2005) Extended rituximab therapy in Waldenstrom’s macroglobulinemia. Ann Oncol 16:132–138CrossRefPubMed
25.
Zurück zum Zitat Weide R, Heymanns J, Koppler H (1999) Induction of complete haematological remission after monotherapy with anti-CD20 monoclonal antibody (RITUXIMAB) in a patient with alkylating agent resistant Waldenstrom’s macroglobulinaemia. Leuk Lymphoma 36:203–206CrossRefPubMed Weide R, Heymanns J, Koppler H (1999) Induction of complete haematological remission after monotherapy with anti-CD20 monoclonal antibody (RITUXIMAB) in a patient with alkylating agent resistant Waldenstrom’s macroglobulinaemia. Leuk Lymphoma 36:203–206CrossRefPubMed
26.
Zurück zum Zitat Weide R, Heymanns J, Koppler H (2000) The polyneuropathy associated with Waldenstrom’s macroglobulinaemia can be treated effectively with chemotherapy and the anti-CD20 monoclonal antibody rituximab. Br J Haematol 109:838–841CrossRefPubMed Weide R, Heymanns J, Koppler H (2000) The polyneuropathy associated with Waldenstrom’s macroglobulinaemia can be treated effectively with chemotherapy and the anti-CD20 monoclonal antibody rituximab. Br J Haematol 109:838–841CrossRefPubMed
27.
Zurück zum Zitat Bing J, Fog M, Neel A (1937) Reports 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 Bing J, Fog M, Neel A (1937) Reports 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
28.
Zurück zum Zitat Tommasi M, Revol L, Schott B, Lesbros F (1966) [Waldenstrom’s macroglobulinemia. Lesions of the central nervous system]. Ann Anat Pathol (Paris) 11:309–313 Tommasi M, Revol L, Schott B, Lesbros F (1966) [Waldenstrom’s macroglobulinemia. Lesions of the central nervous system]. Ann Anat Pathol (Paris) 11:309–313
29.
Zurück zum Zitat Pennacchio L, Orlandini M (1969) [Immunoglobulin diseases. (Basis of modern immunology. Clinical contribution. Bing-Neel syndrome)]. Policlinico Prat 76:401–422PubMed Pennacchio L, Orlandini M (1969) [Immunoglobulin diseases. (Basis of modern immunology. Clinical contribution. Bing-Neel syndrome)]. Policlinico Prat 76:401–422PubMed
30.
Zurück zum Zitat Quilichini R, Benderitter T, Lafeuillade A, Chaffanjon P, Aubert L (1989) [Waldenstrom’s macroglobulinemia with cerebral lymphoproliferative involvement of tumor appearance (Bing and Neel syndrome). An anatomo-clinical case]. Ann Med Interne (Paris) 140:25–29 Quilichini R, Benderitter T, Lafeuillade A, Chaffanjon P, Aubert L (1989) [Waldenstrom’s macroglobulinemia with cerebral lymphoproliferative involvement of tumor appearance (Bing and Neel syndrome). An anatomo-clinical case]. Ann Med Interne (Paris) 140:25–29
31.
Zurück zum Zitat Jammes T, Mann C, Delhom E, Bertrand P, Berthelemy C, Oweini K, Jonquet O (1990) [Bing-Neel syndrome revealed by normal pressure hydrocephalus. Unusual clinical/course of Waldenstrom disease]. Presse Med 19:1992PubMed Jammes T, Mann C, Delhom E, Bertrand P, Berthelemy C, Oweini K, Jonquet O (1990) [Bing-Neel syndrome revealed by normal pressure hydrocephalus. Unusual clinical/course of Waldenstrom disease]. Presse Med 19:1992PubMed
32.
Zurück zum Zitat Shimizu K, Fujisawa K, Yamamoto H, Mizoguchi Y, Hara K (1993) Importance of central nervous system involvement by neoplastic cells in a patient with Waldenstrom’s macroglobulinemia developing neurologic abnormalities. Acta Haematol 90:206–208CrossRefPubMed Shimizu K, Fujisawa K, Yamamoto H, Mizoguchi Y, Hara K (1993) Importance of central nervous system involvement by neoplastic cells in a patient with Waldenstrom’s macroglobulinemia developing neurologic abnormalities. Acta Haematol 90:206–208CrossRefPubMed
33.
Zurück zum Zitat Civit T, Coulbois S, Baylac F, Taillandier L, Auque J (1997) [Waldenstrom’s macroglobulinemia and cerebral lymphoplasmocytic proliferation: Bing and Neel syndrome. Apropos of a new case]. Neurochirurgie 43:245–249PubMed Civit T, Coulbois S, Baylac F, Taillandier L, Auque J (1997) [Waldenstrom’s macroglobulinemia and cerebral lymphoplasmocytic proliferation: Bing and Neel syndrome. Apropos of a new case]. Neurochirurgie 43:245–249PubMed
34.
Zurück zum Zitat Arias M, Pereiro Zabala I, Requena Caballero I, Sesar Ignacio A, Arias Rivas S, Villamayor Blanco B (2004) [Rapidly progressing dementia as the presenting symptom of Waldenstrom’s macroglobulinemia: findings from magnetic resonance imaging of the brain in Bing Neel syndrome]. Rev Neurol 38:640–642PubMed Arias M, Pereiro Zabala I, Requena Caballero I, Sesar Ignacio A, Arias Rivas S, Villamayor Blanco B (2004) [Rapidly progressing dementia as the presenting symptom of Waldenstrom’s macroglobulinemia: findings from magnetic resonance imaging of the brain in Bing Neel syndrome]. Rev Neurol 38:640–642PubMed
35.
Zurück zum Zitat Kim HD, Shin KC, Cho HS, Kim MK, Lee KH, Hyun MS (2007) Therapeutic experience of Bing-Neel syndrome associated with Waldenstrom’s macroglobulinemia. J Korean Med Sci 22:1079–1081CrossRefPubMed Kim HD, Shin KC, Cho HS, Kim MK, Lee KH, Hyun MS (2007) Therapeutic experience of Bing-Neel syndrome associated with Waldenstrom’s macroglobulinemia. J Korean Med Sci 22:1079–1081CrossRefPubMed
36.
Zurück zum Zitat Drappatz J, Akar S, Fisher DC, Samuels MA, Kesari S (2008) Imaging of Bing-Neel syndrome. Neurology 70:1364CrossRefPubMed Drappatz J, Akar S, Fisher DC, Samuels MA, Kesari S (2008) Imaging of Bing-Neel syndrome. Neurology 70:1364CrossRefPubMed
Metadaten
Titel
Bing–Neel syndrome: an illustrative case and a comprehensive review of the published literature
verfasst von
Roneil G. Malkani
Martin Tallman
Numa Gottardi-Littell
William Karpus
Laura Marszalek
Daina Variakojis
Bruce Kaden
Matthew Walker
Robert M. Levy
Jeffrey J. Raizer
Publikationsdatum
01.02.2010
Verlag
Springer US
Erschienen in
Journal of Neuro-Oncology / Ausgabe 3/2010
Print ISSN: 0167-594X
Elektronische ISSN: 1573-7373
DOI
https://doi.org/10.1007/s11060-009-9968-3

Weitere Artikel der Ausgabe 3/2010

Journal of Neuro-Oncology 3/2010 Zur Ausgabe

Leitlinien kompakt für die Neurologie

Mit medbee Pocketcards sicher entscheiden.

Seit 2022 gehört die medbee GmbH zum Springer Medizin Verlag

Neu im Fachgebiet Neurologie

Update Neurologie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.