Preferences help
enabled [disable] Abstract
Number of results
2018 | 108 | 87-98
Article title

An overview of paraneoplastic neurological syndromes – pathophysiology and clinical insight

Title variants
Languages of publication
Paraneoplastic syndrome (PS) is a dysfunction of organs or systems, associated with neoplastic disease, but not related to the local growth of tumor, metastasis or adverse anti-cancer drugs reactions. Neurological paraneoplastic syndromes (NPSs) may affect every region of a human nervous system - both central, peripheral, and/or autonomic nervous system. The symptoms are caused by a neoplastic process in other organ or system. PNSs usually precede the development of cancer for months or even years, and can be therefore useful diagnostic markers of cancer. They present an autoimmune background associated with the response of the immune system against cancer cells. In the blood serum and cerebrospinal fluid of patients with PNS appear onconeural antibodies, reacting with tumor antigens and the brain, the spinal cord and peripheral ganglia antigens. Many authors emphasize the significance of paraneoplastic syndromes in the modern oncology. Due to this reason, the knowledge of paraneoplastic syndromes and their mechanisms is very important in the contemporary medicine. The purpose of this research review is to summarize the information about the clinical features of the most common PNSs and the pathological mechanisms of their development.
Physical description
  • Faculty of Medicine, Wroclaw Medical University, J. Mikulicza-Radeckiego 5, 50-345 Wroclaw, Poland
  • Faculty of Medicine, Wroclaw Medical University, J. Mikulicza-Radeckiego 5, 50-345 Wroclaw, Poland
  • Chair and Department of Pharmacology and Pharmacodynamics, Medical University of Lublin, Chodzki 4a, 20-093, Lublin, Poland
  • Faculty of Medicine, Wroclaw Medical University, J. Mikulicza-Radeckiego 5, 50-345 Wroclaw, Poland
  • Faculty of Medicine, Wroclaw Medical University, J. Mikulicza-Radeckiego 5, 50-345 Wroclaw, Poland
  • Faculty of Biotechnology, University of Wroclaw, Joliot-Curie 14a, 50-385 Wroclaw, Poland
  • Department of Molecular and Cellular Biology, Wroclaw Medical University, Borowska 211A, 50-556, Wroclaw, Poland
  • [1] Pelosof LC, Gerber DE. Paraneoplastic syndromes: an approach to diagnosis and treatment. Mayo Clin Proc 85(9) (2010) 838–54
  • [2] Leypoldt F, Wandinger KP. Paraneoplastic neurological syndromes. Clin Exp Immunol 175(3) (2014) 336–48
  • [3] Honnorat J, Antoine JC. Paraneoplastic neurological syndromes. Orphanet J Rare Dis 2(1) (2007) 22
  • [4] Graus F, Dalmau J. Paraneoplastic neurological syndromes. Curr Opin Neurol 25(6) (2012) 795–801
  • [5] Kyaw H, Shaikh AZ, Ayala-Rodriguez C, Deepika M. Paraneoplastic Cardiac Involvement in Renal Cell Carcinoma With Dermatomyositis Sine Dermatitis. Ochsner J 17(4) (2017) 421–5
  • [6] Kannoth S. Paraneoplastic neurologic syndrome: A practical approach. Ann Indian Acad Neurol 15(1) (2012) 6–12
  • [7] Eichmüller SB, Bazhin A V. Onconeural versus paraneoplastic antigens? Curr Med Chem 14(23) (2007) 2489–94
  • [8] Dalmau J, Gultekin HS, Posner JB. Paraneoplastic neurologic syndromes: pathogenesis and physiopathology. Brain Pathol 9(2) (1999) 275–84
  • [9] McKeon A, Pittock SJ. Paraneoplastic encephalomyelopathies: pathology and mechanisms. Acta Neuropathol 122(4) (2011) 381–400
  • [10] Storstein A, Vedeler CA. Paraneoplastic neurological syndromes and onconeural antibodies: clinical and immunological aspects. Adv Clin Chem 44 (2007) 143–85
  • [11] Raspotnig M, Vedeler CA, Storstein A. Onconeural antibodies in patients with neurological symptoms: detection and clinical significance. Acta Neurol Scand 124(191) (2011) 83–8
  • [12] Graus F, Dalmau J. Paraneoplastic neurological syndromes. Curr Opin Neurol 25(6) (2012) 795–801
  • [13] Romi F. Thymoma in myasthenia gravis: from diagnosis to treatment. Autoimmune Dis 2011 (2011) 474512
  • [14] Bukhari S, Soomro R, Fawwad S, Alvarez C, Wallach S. Adenocarcinoma of Lung Presenting as Lambert-Eaton Myasthenic Syndrome. J Investig Med high impact case reports 5(3) (2017) 2324709617721251
  • [15] Rothenberg AB, Berdon WE, D’Angio GJ, Yamashiro DJ, Cowles RA. The association between neuroblastoma and opsoclonus-myoclonus syndrome: a historical review. Pediatr Radiol 39(7) (2009) 723–6
  • [16] Klaas JP, Ahlskog JE, Pittock SJ, Matsumoto JY, Aksamit AJ, Bartleson JD, et al. Adult-Onset Opsoclonus-Myoclonus Syndrome. Arch Neurol 69(12) (2012) 1598
  • [17] Bishay RH, Paton J, Abraham V. Variant Guillain-Barré Syndrome in a Patient with Non-Hodgkin’s Lymphoma. Case Rep Hematol 2015 (2015) 979237
  • [18] Honnorat J. Onconeural antibodies are essential to diagnose paraneoplastic neurological syndromes. Acta Neurol Scand 113(s183) (2006) 64–8
  • [19] Rosenfeld MR, Dalmau J. Paraneoplastic Neurologic Disorders: A Brief Overview. Memo 5(3) (2012) 197–200
  • [20] Michalak S, Cofta S, Piatek A, Rybacka J, Wysocka E, Kozubski W. Onconeuronal and antineuronal antibodies in patients with neoplastic and non-neoplastic pulmonary pathologies and suspected for paraneoplastic neurological syndrome. Eur J Med Res 14 Suppl 4 (2009) 156–61
  • [21] Psimaras D, Carpentier AF, Rossi C, PNS Euronetwork. Cerebrospinal fluid study in paraneoplastic syndromes. J Neurol Neurosurg Psychiatry 81(1) (2010) 42–5
  • [22] Blaes F. Paraneoplastic neurological syndromes--diagnosis and management. Curr Pharm Des 18(29) (2012) 4518–25
  • [23] Yuasa T, Fujita K. Limbic encephalitis - history, symptoms and the latest classification. Brain Nerve 62(8) (2010) 817–26
  • [24] Dalmau J, Lancaster E, Martinez-Hernandez E, Rosenfeld MR, Balice-Gordon R. Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. Lancet Neurol 10(1) (2011) 63–74
  • [25] Alamowitch S, Graus F, Uchuya M, Reñé R, Bescansa E, Delattre JY. Limbic encephalitis and small cell lung cancer. Clinical and immunological features. Brain 120 (Pt6) (1997) 923–8
  • [26] Bakheit AM, Kennedy PG, Behan PO. Paraneoplastic limbic encephalitis: clinico-pathological correlations. J Neurol Neurosurg Psychiatry 53(12) (1990) 1084–8
  • [27] Gultekin SH, Rosenfeld MR, Voltz R, Eichen J, Posner JB, Dalmau J. Paraneoplastic limbic encephalitis: neurological symptoms, immunological findings and tumour association in 50 patients. Brain 123(7) (2000) 1481–94
  • [28] Schmitt SE, Pargeon K, Frechette ES, Hirsch LJ, Dalmau J, Friedman D. Extreme delta brush: a unique EEG pattern in adults with anti-NMDA receptor encephalitis. Neurology 79(11) (2012) 1094–100
  • [29] Rojas I, Graus F, Keime-Guibert F, Reñé R, Delattre JY, Ramón JM, et al. Long-term clinical outcome of paraneoplastic cerebellar degeneration and anti-Yo antibodies. Neurology 55(5) (2000) 713–5
  • [30] Shams’ili S, Grefkens J, de Leeuw B, van den Bent M, Hooijkaas H, van der Holt B, et al. Paraneoplastic cerebellar degeneration associated with antineuronal antibodies: analysis of 50 patients. Brain 126(6) (2003) 1409–18
  • [31] Afzal S, Recio M, Shamim S. Paraneoplastic cerebellar ataxia and the paraneoplastic syndromes. Proc (Bayl Univ Med Cent) 28(2) (2015) 217–20
  • [32] Greenlee JE. Treatment of Paraneoplastic Cerebellar Degeneration. Curr Treat Options Neurol 15(2) (2013) 185–200
  • [33] Suri V, Khan NI, Jadhao N, Gupta R. Paraneoplastic cerebellar degeneration in Hodgkin’s lymphoma. Ann Indian Acad Neurol 15(3) (2012) 205–7
  • [34] Zhu Y, Chen S, Chen S, Song J, Chen F, Guo H, et al. An uncommon manifestation of paraneoplastic cerebellar degeneration in a patient with high grade urothelial, carcinoma with squamous differentiation: A case report and literature review. BMC Cancer 16 (2016) 324
  • [35] Hammack JE, Kimmel DW, O’Neill BP, Lennon VA. Paraneoplastic cerebellar degeneration: a clinical comparison of patients with and without Purkinje cell cytoplasmic antibodies. Mayo Clin Proc 65(11) (1990) 1423–31
  • [36] Rees JH. Paraneoplastic cerebellar degeneration: new insights into imaging and immunology. J Neurol Neurosurg Psychiatry 77(4) (2006) 427
  • [37] Elomrani F, Ouziane I, Boutayeb S, Bensouda Y, Mrabti H, Errihani H. Ovarian cancer revealed by paraneoplastic cerebellar degeneration: a case report. Pan Afr Med J 18 (2014) 2
  • [38] Adama D, Moussa B, Emmanuel M, Dennis U. Breast cancer revealed by a paraneoplastic cerebellar syndrome: about one case and literature review. Pan Afr Med J 22 (2015) 25
  • [39] Rudnick E, Khakoo Y, Antunes NL, Seeger RC, Brodeur GM, Shimada H, et al. Opsoclonus-myoclonus-ataxia syndrome in neuroblastoma: Clinical outcome and antineuronal antibodies - a report from the children’s cancer group study. Med Pediatr Oncol 36(6) (2001) 612–22
  • [40] Deconinck N, Scaillon M, Segers V, Groswasser JJ, Dan B. Opsoclonus-Myoclonus Associated With Celiac Disease. Pediatr Neurol 34(4) (2006) 312–4
  • [41] Sahu JK, Prasad K. The opsoclonus-myoclonus syndrome. Pract Neurol 11(3) (2011) 160–6
  • [42] Scarff JR, Iftikhar B, Tatugade A, Choi J, Lippmann S. Opsoclonus myoclonus. Innov Clin Neurosci 8(12) (2011) 29–31
  • [43] Koziorowska-Gawron E, Koszewicz M, Budrewicz S. Zespół opsoklonie – mioklonie u dorosłych. Oficjalne Portale Internetowe PTN 1942 (2014) 101–5
  • [44] Caviness JN, Truong DD. Myoclonus. In: Handbook of clinical neurology (2011) 399–420
  • [45] Jasminekalyani P, Saravanan S. Dancing eyes dancing feet syndrome-a report of two cases. J Clin Diagn Res 8(5) (2014) MD03-5
  • [46] Bataller L, Graus F, Saiz A, Vilchez JJ, Spanish Opsoclonus-Myoclonus Study Group. Clinical outcome in adult onset idiopathic or paraneoplastic opsoclonus-myoclonus. Brain 124(Pt 2) (2001) 437–43
  • [47] Gorman MP. Update on diagnosis, treatment, and prognosis in opsoclonus–myoclonus–ataxia syndrome. Curr Opin Pediatr 22(6) (2010) 745–50
  • [48] Weizman DA, Leong WL. Anti-Ri antibody opsoclonus-myoclonus syndrome and breast cancer: A case report and a review of the literature. J Surg Oncol 87(3) (2004) 143–5
  • [49] Lee JH, Shin JH, Kim DS, Jung DS, Park KH, Lee MK, et al. A case of Lambert-Eaton myasthenic syndrome associated with atypical bronchopulmonary carcinoid tumor. J Korean Med Sci 19(5) (2004) 753–5
  • [50] Gutmann L, Phillips LH, Gutmann L. Trends in the association of Lambert-Eaton myasthenic syndrome with carcinoma. Neurology 42(4) (1992) 848–50
  • [51] Zambelis T, Foutsitzi A, Giannakopoulou A, Poulopoulou K, Karandreas N. Lambert-Eaton myasthenic syndrome. Clinical and electrophysiological findings in seven cases. Electromyogr Clin Neurophysiol 44(5) (2004) 289–92
  • [52] Oh SJ, Hatanaka Y, Ito E, Nagai T. Post-exercise exhaustion in Lambert–Eaton myasthenic syndrome. Clin Neurophysiol 125(2) (2014) 411–4
  • [53] Gilhus NE. Lambert-eaton myasthenic syndrome; pathogenesis, diagnosis, and therapy. Autoimmune Dis 2011 (2011) 973808
  • [54] Nicolle MW. Myasthenia Gravis and Lambert-Eaton Myasthenic Syndrome. Contin Lifelong Learn Neurol 22(6) (2016) 1978–2005
  • [55] Marvi U, Chung L, Fiorentino DF. Clinical presentation and evaluation of dermatomyositis. Indian J Dermatol 57(5) (2012) 375–81
  • [56] Findlay AR, Goyal NA, Mozaffar T. An overview of polymyositis and dermatomyositis. Muscle Nerve 51(5) (2015) 638–56
Document Type
Publication order reference
YADDA identifier
JavaScript is turned off in your web browser. Turn it on to take full advantage of this site, then refresh the page.