SÍNDROMES PARANEOPLÁSICOS DEL SISTEMA NERVIOSO CENTRAL

Lenin Almanza S.; Ana Belén Araúz R.

Submitted October 13, 2024

Published 2026-04-06

Artículos de Investigación

Vol. 17 No. 1 (2004): Revista Médico Científica

PARANEOPLASTIC SYNDROMES OF THE CENTRAL NERVOUS SYSTEM

Lenin Almanza S.⁺⁻
Ana Belén Araúz R.⁺⁻

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Published: 2026-04-06

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Abstract

Paraneoplastic syndromes are defined as the remote effects of malignancy that cannot be attributed either to direct invasion or metastasic lesions. These syndromes may be the first sign of a malignancy (early detection) and some of them may constitute a more urgent hazard to life than the underlying cancer, which is why they are important to the clinician.

These syndromes have been classified according to the effects they may produce in endocrinologic, dermatologic, hemawlogic, metabolic or neuromusculars. In this review we will concentrate in those related with the central nervous system because they are frequently overlooked by the physician.

Neuronal paraneoplastic syndromes have been found to be caused by the production of antineuronal antibodies, since tumoral antigens are similar to neuronal they involve in a cross-reactive antibody. We will emphasize in those antibodies described in the literature. The paraneoplastic syndromes will be review according to clinical presentation, pathology and prognostic aspects.

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References

Rosenfeld M, Dalmau J. The clinical spectrum and pathogenesis of paraneoplastic disorders of the central nervous system. Hematol Oncol Clin North Am 2001; 15(6): 1109-28.
Hinton R. Paraneoplastic neurologic syndromes. Hematol Oncol Clin North Am 1996; 10(4): 909-25.
Dalmau J, Furneaux H, Gralla AJ. Detection of the anti-Hu antibody in the serum of patients with small cell lung cancer—a quantitative Western blot analysis. Ann Neurol 1990; 27:544-52.
Furneaux H, Rosenblum M, Dalmau J. Selective expression of Purkinje-cell antigen in tumor tissue from patient with paraneoplastic cerebellar degeneration. N Engl J Med 1990; 322:1844-51.
Furneaux H, Aeich L, Posner J. Autoantibody synthesis in the central nervous system of patients with paraneoplastic syndromes. Neurology 1990; 40:1085-91.
Anderson N, Rosenblum M, Graus F. Autoantibodies in paraneoplastic syndromes associated with small-cell lung cancer. Neurology 1988; 38:1391-98.
Benyahia B, Liblau R, Merle-Beral H. Cell-mediated autoimmunity in paraneoplastic neurologic syndromes with anti-Hu antibodies. Ann Neurol 1999; 45:162-7.
Dalmau J, Graus F, Cheung N. Major histocompatibility proteins, anti-Hu antibodies, and paraneoplastic encephalomyelitis in neuroblastoma and small cell lung cancer. Cancer 1995; 75:99-109.
Darnell R, DeAngelis L. Regression of small-cell lung carcinoma in patients with paraneoplastic neuronal antibodies. Lancet 1993; 341: 21-2.
Graus F, Ribalta T, Campo E. Immunohistochemical analysis of the immune reaction in the nervous system in paraneoplastic encephalomyelitis. Neurology 1990; 40:219-22.
Forsyth P, Dalmau J, Graus F. Motor neuron syndromes in cancer patients. Ann Neurol 1997; 41:722-30.
Dalmau J, Rosenfeld M. Paraneoplastic syndromes of the nervous system. UpToDate CD-Rom. 2001; 9(3).
Dalmau J, Graus F, Rosenblum M. Anti-Hu-associated paraneoplastic encephalomyelitis/sensory neuropathy: A clinical study of 71 patients. Medicine 1992; 71:59-72.
Dalmau J, Gultekin SH, Voltz R, Hoard R, DesChamps T, Balmaceda C, et al. Ma1, a novel neuron- and testis-specific protein, is recognized by the serum of patients with paraneoplastic neurological disorders. Brain 1999; 122(Pt 1):27-39.
Zacharias A, Brashear H, Muñoz E. Paraneoplastic encephalomyelitis obscured by coma. Neurology 1996; 46:1177-9.
Voltz R, Gultekin S, Rosenfeld M. A serologic marker of paraneoplastic limbic and brain-stem encephalitis in patients with testicular cancer. N Engl J Med 1999; 340:1788-95.
Pillay N, Gilbert J, Ebers G, Brown J. Internuclear ophthalmoplegia and "optic neuritis": paraneoplastic effects of bronchial carcinoma. Neurology 1984; 34:788-91.
Peterson K, Rosenblum M, Kotanides H. Paraneoplastic cerebellar degeneration: A clinical analysis of 55 anti-Yo antibody positive patients. Neurology 1992; 42:1931-7.
Anderson N, Rosenblum M, Posner J. Paraneoplastic cerebellar degeneration: Clinical-immunological correlations. Ann Neurol 1988; 24:559-67.
Trotter J, Hendin B, Osterland K. Cerebellar degeneration with Hodgkin's disease: An immunological study. Arch Neurol 1976; 33:660-1.
Sillevis S, Kinoshita A, De Leeuw B, Moll W, Coesmans M, Jaarsma D, et al. Paraneoplastic cerebellar ataxia due to autoantibodies against a glutamate receptor. N Engl J Med 2000; 342:21-7.
Antoine J, Honnorat J, Camdessanche J, Magistris M, Absi L, Mosnier J, et al. Paraneoplastic anti-GV2 antibodies react with peripheral nerve and are associated with a mixed axonal and demyelinating peripheral neuropathy. Ann Neurol 2001; 49:214-21.
Saiz A, Graus F, Dalmau J, Pifarre A, Marin C, Tolosa E. Detection of 14-3-3 brain protein in the cerebrospinal fluid of patient with paraneoplastic neurological disorders. Ann Neurol 1999; 46:774-7.
Digre K. Opsoclonus in adults. Report of three cases and review of the literature. Arch Neurol 1986; 43:1165-75.
Anderson N, Budde-Steffen C, Rosenblum M. Opsoclonus, myoclonus, ataxia, and encephalopathy in adults with cancer: A distinct paraneoplastic syndrome. Medicine 1988; 67:100-9.
Dropcho E, Payne A. Paraneoplastic opsoclonus-myoclonus: Association with medullary thyroid carcinoma and review of the literature. Arch Neurol 1986; 43:410-5.
Jacobson O, Thirkill C, Tipping S. A clinical triad to diagnose paraneoplastic retinopathy. Ann Neurol 1990; 28:162-7.
Campo E, Brunier M, Merino M. Small cell carcinoma of the endometrium with associated ocular paraneoplastic syndrome. Cancer 1992; 69:2283-8.
Thirkill C, FitzGerald P, Sergott A, Roth A, Tyler N, Keltner J. Cancer-associated retinopathy (CAR syndrome) with antibodies reacting with retinal, optic-nerve, and cancer cells. N Engl J Med 1989; 321:1589-94.
Ojeda V. Necrotizing myelopathy associated with malignancy. A clinicopathologic study of two cases and literature review. Cancer 1984; 53:1115-23.
Shaw P. Stiff-man syndrome and its variants. Lancet 1999; 353:86-7.
Solimena M, Folli F, Aparisi R. Autoantibodies to GABA-ergic neurons and pancreatic beta cells in stiff-man syndrome. N Engl J Med 1990; 322:1550-60.
Folli F, Solimena M, Cofiell R. Autoantibodies to a 128-kd synaptic protein in three women with the stiff-man syndrome and breast cancer. N Engl J Med 1993; 328:546-51.

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[1] Rosenfeld M, Dalmau J. The clinical spectrum and pathogenesis of paraneoplastic disorders of the central nervous system. Hematol Oncol Clin North Am 2001; 15(6): 1109-28.

[2] Hinton R. Paraneoplastic neurologic syndromes. Hematol Oncol Clin North Am 1996; 10(4): 909-25.

[3] Dalmau J, Furneaux H, Gralla AJ. Detection of the anti-Hu antibody in the serum of patients with small cell lung cancer—a quantitative Western blot analysis. Ann Neurol 1990; 27:544-52.

[4] Furneaux H, Rosenblum M, Dalmau J. Selective expression of Purkinje-cell antigen in tumor tissue from patient with paraneoplastic cerebellar degeneration. N Engl J Med 1990; 322:1844-51.

[5] Furneaux H, Aeich L, Posner J. Autoantibody synthesis in the central nervous system of patients with paraneoplastic syndromes. Neurology 1990; 40:1085-91.

[6] Anderson N, Rosenblum M, Graus F. Autoantibodies in paraneoplastic syndromes associated with small-cell lung cancer. Neurology 1988; 38:1391-98.

[7] Benyahia B, Liblau R, Merle-Beral H. Cell-mediated autoimmunity in paraneoplastic neurologic syndromes with anti-Hu antibodies. Ann Neurol 1999; 45:162-7.

[8] Dalmau J, Graus F, Cheung N. Major histocompatibility proteins, anti-Hu antibodies, and paraneoplastic encephalomyelitis in neuroblastoma and small cell lung cancer. Cancer 1995; 75:99-109.

[9] Darnell R, DeAngelis L. Regression of small-cell lung carcinoma in patients with paraneoplastic neuronal antibodies. Lancet 1993; 341: 21-2.

[10] Graus F, Ribalta T, Campo E. Immunohistochemical analysis of the immune reaction in the nervous system in paraneoplastic encephalomyelitis. Neurology 1990; 40:219-22.

[11] Forsyth P, Dalmau J, Graus F. Motor neuron syndromes in cancer patients. Ann Neurol 1997; 41:722-30.

[12] Dalmau J, Rosenfeld M. Paraneoplastic syndromes of the nervous system. UpToDate CD-Rom. 2001; 9(3).

[13] Dalmau J, Graus F, Rosenblum M. Anti-Hu-associated paraneoplastic encephalomyelitis/sensory neuropathy: A clinical study of 71 patients. Medicine 1992; 71:59-72.

[14] Dalmau J, Gultekin SH, Voltz R, Hoard R, DesChamps T, Balmaceda C, et al. Ma1, a novel neuron- and testis-specific protein, is recognized by the serum of patients with paraneoplastic neurological disorders. Brain 1999; 122(Pt 1):27-39.

[15] Zacharias A, Brashear H, Muñoz E. Paraneoplastic encephalomyelitis obscured by coma. Neurology 1996; 46:1177-9.

[16] Voltz R, Gultekin S, Rosenfeld M. A serologic marker of paraneoplastic limbic and brain-stem encephalitis in patients with testicular cancer. N Engl J Med 1999; 340:1788-95.

[17] Pillay N, Gilbert J, Ebers G, Brown J. Internuclear ophthalmoplegia and "optic neuritis": paraneoplastic effects of bronchial carcinoma. Neurology 1984; 34:788-91.

[18] Peterson K, Rosenblum M, Kotanides H. Paraneoplastic cerebellar degeneration: A clinical analysis of 55 anti-Yo antibody positive patients. Neurology 1992; 42:1931-7.

[19] Anderson N, Rosenblum M, Posner J. Paraneoplastic cerebellar degeneration: Clinical-immunological correlations. Ann Neurol 1988; 24:559-67.

[20] Trotter J, Hendin B, Osterland K. Cerebellar degeneration with Hodgkin's disease: An immunological study. Arch Neurol 1976; 33:660-1.

[21] Sillevis S, Kinoshita A, De Leeuw B, Moll W, Coesmans M, Jaarsma D, et al. Paraneoplastic cerebellar ataxia due to autoantibodies against a glutamate receptor. N Engl J Med 2000; 342:21-7.

[22] Antoine J, Honnorat J, Camdessanche J, Magistris M, Absi L, Mosnier J, et al. Paraneoplastic anti-GV2 antibodies react with peripheral nerve and are associated with a mixed axonal and demyelinating peripheral neuropathy. Ann Neurol 2001; 49:214-21.

[23] Saiz A, Graus F, Dalmau J, Pifarre A, Marin C, Tolosa E. Detection of 14-3-3 brain protein in the cerebrospinal fluid of patient with paraneoplastic neurological disorders. Ann Neurol 1999; 46:774-7.

[24] Digre K. Opsoclonus in adults. Report of three cases and review of the literature. Arch Neurol 1986; 43:1165-75.

[25] Anderson N, Budde-Steffen C, Rosenblum M. Opsoclonus, myoclonus, ataxia, and encephalopathy in adults with cancer: A distinct paraneoplastic syndrome. Medicine 1988; 67:100-9.

[26] Dropcho E, Payne A. Paraneoplastic opsoclonus-myoclonus: Association with medullary thyroid carcinoma and review of the literature. Arch Neurol 1986; 43:410-5.

[27] Jacobson O, Thirkill C, Tipping S. A clinical triad to diagnose paraneoplastic retinopathy. Ann Neurol 1990; 28:162-7.

[28] Campo E, Brunier M, Merino M. Small cell carcinoma of the endometrium with associated ocular paraneoplastic syndrome. Cancer 1992; 69:2283-8.

[29] Thirkill C, FitzGerald P, Sergott A, Roth A, Tyler N, Keltner J. Cancer-associated retinopathy (CAR syndrome) with antibodies reacting with retinal, optic-nerve, and cancer cells. N Engl J Med 1989; 321:1589-94.

[30] Ojeda V. Necrotizing myelopathy associated with malignancy. A clinicopathologic study of two cases and literature review. Cancer 1984; 53:1115-23.

[31] Shaw P. Stiff-man syndrome and its variants. Lancet 1999; 353:86-7.

[32] Solimena M, Folli F, Aparisi R. Autoantibodies to GABA-ergic neurons and pancreatic beta cells in stiff-man syndrome. N Engl J Med 1990; 322:1550-60.

[33] Folli F, Solimena M, Cofiell R. Autoantibodies to a 128-kd synaptic protein in three women with the stiff-man syndrome and breast cancer. N Engl J Med 1993; 328:546-51.

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