Paraneoplastic Neurologic Syndromes

Paraneoplastic neurological syndromes (PNS) are diseases that occur due to the remote effects of tumors (usually malignant). The most common tumors associated with PNS include small cell lung cancer (SCLC), thymoma, neuroblastoma, ovarian, breast, testicular, and Hodgkin lymphoma. Researchers believe PNS is caused by cancerfighting abilities of the immune system, in particular antibodies and specific white blood cells, known as T- cells. Instead of attacking only the cancer cells, these immune agents also attack the normal cells of the nervous system and cause neurological disorders.





Test Information

Test Number Test Name
2013955 Paraneoplastic Reflexive Panel
2013944 Autoimmune Neurologic Disease Reflexive Panel
2013601 Autoimmune Encephalitis Reflexive Panel
2005640 Autoimmune Neuromuscular Junction Reflexive Panel

When to Order Testing

  • Neurologic disease of unknown etiology without evidence of malignancy
  • Neurologic disease with high suspicion of malignancy or known risk factors for malignancy

Why is Testing Important?

  • Aids in the diagnosis of paraneoplastic neurological disease associated with certain cancers (carcinoma of lung, breast, ovary, thymoma, or Hodgkin’s lymphoma) and/or related disorders such as autoimmune encephalitis
  • Directs a focused search for cancer
  • Differentiates autoimmune neuropathies from neurotoxic effects of chemotherapy
  • Monitors the immune response of seropositive patients in the course of cancer therapy
  • Can detect early evidence of cancer recurrence in previously seropositive patients

Benefits of PNS Testing at ARUP

  • ARUP offers a comprehensive list of tests that represent the autoantibodies found in about 85-90% of patients with PNS or other autoimmune neurologic disorder (as standalone or in panels).
  • ARUP Panels are directed at specific syndromes allowing for more targeted testing than the large panels offered by competitors.
  • Smaller directed panels and individual tests are more cost effective.

Additional Resources

Guidelines

  • Graus F, Delattre JY, Antoine JC, Dalmau J, Giometto B, Grisold W, Honnorat J, Smitt S, Vedeler C, Verschuuren JJ, Vincent A, Voltz R. Recommended diagnostic criteria for paraneoplastic neurological syndromes. J Neurol Neurosurg Psychiatry. 2004; 75(8): 1135-40. PubMed
  • Titulaer MJ, Soffietti R, Dalmau J, Gilhus NE, Giometto B, Graus F, Grisold W, Honnorat J, Smitt PA, Tanasescu R, Vedeler CA, Voltz R, Verschuuren JJ, European Federation of Neurological Societies. Screening for tumours in paraneoplastic syndromes: report of an EFNS task force. Eur J Neurol. 2011; 18(1): 19-e3. PubMed
  • Zuliani L, Graus F, Giometto B, Bien C, Vincent A. Central nervous system neuronal surface antibody associated syndromes: review and guidelines for recognition. J Neurol Neurosurg Psychiatry. 2012; 83(6): 638-45. PubMed

References

  • Horta ES, Lennon VA, Lachance DH, Jenkins SM, Smith CY, McKeon A, Klein C, Pittock SJ. Neural autoantibody clusters aid diagnosis of cancer. Clin Cancer Res. 2014 Jul 15;20(14):3862–9.
  • Tebo AE, Haven TR, Jackson BR. Autoantibody diversity in paraneoplastic syndromes and related disorders: The need for a more guided screening approach. Clin Chim Acta. 2016 Aug 1;459:162–9.
  • Didelot A, Honnorat J. Paraneoplastic disorders of the central and peripheral nervous system. In J Biller and JM Ferro, eds. Neurologic aspects of systemic disease, part III, volume 121 (handbook of clinical neurology), 1st ed. Amsterdam: Elsevier; 2014:1159–79.