Skip to main content

Advertisement

SpringerLink
Log in

Amyotrophic lateral sclerosis with coexisting cancer: a single-center study

  • Original article
  • Published:
Acta Neurologica Belgica Aims and scope Submit manuscript

Abstract

This study investigated the occurrence of malignancy in a cohort of patients with amyotrophic lateral sclerosis (ALS). Forty-three consecutive ALS patients (mean age 61.3 years, 31 men/12 women) admitted between 2015 and 2019 were enrolled. Clinical, electrophysiologic and outcome features that differentiate cancer-associated ALS from classical ALS were investigated. ALS was associated with cancer in 5 (11.6%) patients. Two patients were diagnosed with non-small cell lung, one with endometrium, one with prostate and one with laryngeal carcinoma. One another patient was diagnosed with monoclonal gammopathy of unknown significance, but she did not develop cancer within 12-month follow-up duration. Two patients had a previous diagnosis of cancer before the diagnosis of ALS. In the remaining 3 patients, cancer was diagnosed at the same time or after the onset of ALS. There was no significant difference with respect to mean age of onset, sex, disease duration, clinical and electrophysiological features and outcome between patients with and without malignancy. None of the patients with cancer survived more than two years after the onset of ALS except one who was diagnosed with flail-leg syndrome before the diagnosis of non-small cell lung carcinoma. ALS is not accepted as a classical paraneoplastic neurological syndrome. Although the association of ALS and cancer seems coincidental, a careful investigation for an underlying cancer is important in patients with ALS. In our patient group with cancer-associated ALS, no distinguishing features were identified.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Raymond J, Oskarsson B, Mehta P, Horton K (2019) Clinical characteristics of a large cohort of US participants enrolled in the National Amyotrophic Lateral Sclerosis (ALS) Registry, 2010–2015. Amyotroph Lateral Scler Frontotemporal Degener 20:413–420

    Article  Google Scholar 

  2. Giometto B, Grisold W, Vitaliani R, Graus F, Honnorat J, Bertolini G, Euronetwork PNS (2010) Paraneoplastic neurologic syndrome in the PNS Euronetwork database: a European study from 20 centers. Arch Neurol 67:330–335

    Article  Google Scholar 

  3. Gibson SB, Abbott D, Farnham JM, Thai KK, McLean H, Figueroa KP et al (2016) Population-based risks for cancer in patients with ALS. Neurology 87:289–294

    Article  Google Scholar 

  4. Forsyth PA, Dalmau J, Graus F, Cwik V, Rosenblum MK, Posner JB (1997) Motor neuron syndromes in cancer patients. Ann Neurol 41:722–730

    Article  CAS  Google Scholar 

  5. Sadot E, Carluer L, Corcia P, Delozier Y, Levy C, Viader F (2007) Breast cancer and motor neuron disease: clinical study of seven cases. Amyotroph Lateral Scler 8:288–291

    Article  Google Scholar 

  6. Spataro R, La Bella V (2014) Paraneoplastic motor neuron disease associated with breast cancer. Eur J Neurol 21:e5–6

    Article  CAS  Google Scholar 

  7. Mélé N, Berzero G, Maisonobe T, Salachas F, Nicolas G, Weiss N et al (2018) Motor neuron disease of paraneoplastic origin: a rare but treatable condition. J Neurol 265:1590–1599

    Article  Google Scholar 

  8. Flanagan EP, Sandroni P, Pittock SJ, Inwards DJ, Jones LK Jr (2012) Paraneoplastic lower motor neuronopathy associated with Hodgkin lymphoma. Muscle Nerv 46:823–827

    Article  Google Scholar 

  9. Verschueren A, Gallard J, Boucraut J, Honnorat J, Pouget J, Attarian S (2015) Paraneoplastic subacute lower motor neuron syndrome associated with solid cancer. J Neurol Sci 358:413–416

    Article  Google Scholar 

  10. Brooks BR, Miller RG, Swash M, Munsat TL, World Federation of Neurology Research Group on Motor Neuron Diseases (2000) El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord 1:293–299

    Article  CAS  Google Scholar 

  11. Wijesekera LC, Mathers S, Talman P, Galtrey C, Parkinson MH, Ganesalingam J et al (2009) Natural history and clinical features of the flail arm and flail leg ALS variants. Neurology 72:1087–1094

    Article  CAS  Google Scholar 

  12. Gargiulo-Monachelli GM, Janota F, Bettini M, Shoesmith CL, Strong MJ, Sica RE (2012) Regional spread pattern predicts survival in patients with sporadic amyotrophic lateral sclerosis. Eur J Neurol 19:834–841

    Article  CAS  Google Scholar 

  13. de Carvalho M, Dengler R, Eisen A, England JD, Kaji R, Kimura J (2008) Electrodiagnostic criteria for diagnosis of ALS. Clin Neurophysiol 119:497–503

    Article  Google Scholar 

  14. Cedarbaum JM, Stambler N, Malta E, Fuller C, Hilt D, Thurmond B (1999) The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS Study Group [Phase III]. J Neurol Sci 169:13–21

    Article  CAS  Google Scholar 

  15. Abraham A, Drory VE (2013) Postradiation lower motor neuron syndrome: case series and literature review. J Neurol 260:1802–1806

    Article  CAS  Google Scholar 

  16. Yildiz FG, Bekircan-Kurt CE, Varli K (2017) Single-pulse transcranial magnetic stimulation in amyotrophic lateral sclerosis: experience of a single institution. Neurol Sci Neurophysiol 34:70–75

    Google Scholar 

  17. Vigliani MC, Polo P, Chiò A, Giometto B, Mazzini L, Schiffer D (2000) Patients with amyotrophic lateral sclerosis and cancer do not differ clinically from patients with sporadic amyotrophic lateral sclerosis. J Neurology 247:778–782

    Article  CAS  Google Scholar 

  18. Al-Bustani N, Simonson W, Marshall DA, Vetrovs J, Wener MH, Weiss MD et al (2015) Utility of paraneoplastic antibody testing in the diagnosis of motor neuron disease. J Clin Neuromuscul Dis 17:63–68

    Article  Google Scholar 

  19. Stich O, Kleer B, Rauer S (2007) Absence of paraneoplastic antineuronal antibodies in sera of 145 patients with motor neuron disease. J Neuro Neurosurg Psychiatry 78:883–885

    Article  Google Scholar 

  20. EFNS Task Force on Diagnosis and Management of Amyotrophic Lateral Sclerosis; Andersen PM, Abrahams S, Borasio GD, de Carvalho M, Chio A, Van Damme P, et al (2012) EFNS guidelines on the clinical management of amyotrophic lateral sclerosis [MALS]—revised report of an EFNS task force. Eur J Neurol 19:360–375

  21. Corcia P, Gordon PH, Camdessanche JP (2015) Is there a paraneoplastic ALS? Amyotroph Lateral Scler Frontotemporal Degener 16:252–257

    Article  CAS  Google Scholar 

  22. Fang F, Al-Chalabi A, Ronnevi LO, Turner MR, Wirdefeldt K, Kamel F, Ye W (2013) Amyotrophic lateral sclerosis and cancer: a register-based study in Sweden. Amyotroph Lateral Sclerosis Frontotemporal Degener 14:362–368

    Article  Google Scholar 

  23. Freedman DM, Curtis RE, Daugherty SE, Goedert JJ, Kuncl RW, Tucker MA (2013) The association between cancer and amyotrophic lateral sclerosis. Cancer Causes Control 24:55–60

    Article  Google Scholar 

  24. Verma A, Berger JR, Snodgrass S, Petito C (1996) Motor neuron disease: a paraneoplastic process associated with anti-hu antibody and small-cell lung carcinoma. Ann Neurol 40:112–116

    Article  CAS  Google Scholar 

  25. Gordon PH, Rowland LP, Younger DS, Sherman WH, Hays AP, Louis ED (1997) Lymphoproliferative disorders and motor neuron disease: an update. Neurology 48:1671–1678

    Article  CAS  Google Scholar 

  26. Forman D, Rae-Grant AD, Matchett SC, Cowen JS (1999) A reversible cause of hypercapnic respiratory failure: lower motor neuronopathy associated with renal cell carcinoma. Chest 115:899–901

    Article  CAS  Google Scholar 

  27. Distad BJ, Weiss MD (2010) Paraneoplastic motor neuron disease associated with Purkinje cell autoantibody type 1. J Clin Neuromuscul Dis 12:36–41

    Article  Google Scholar 

  28. Diamanti L, Quaquarini E, Berzero G, Bini P, Gastaldi M, Franciotta D et al (2018) Lower motor neuron syndrome in a patient with HER2-positive metastatic breast cancer: a case report and review of the literature. Clin Neurol Neurosurg 172:141–142

    Article  Google Scholar 

  29. Antoine JC, Camdessanché JP (2017) Paraneoplastic neuropathies. Curr Opin Neurol 30:513–520

    Article  CAS  Google Scholar 

  30. Antoine JC (2014) Peripheral neuropathies associated with antibodies directed to intracellular neural antigens. Rev Neurol [Paris] 170:570–576

    Article  Google Scholar 

  31. Uchuya M, Graus F, Vega F, Reñé R, Delattre JY (1996) Intravenous immunoglobulin treatment in paraneoplastic neurological syndromes with antineuronal autoantibodies. J Neurol Neurosurg Psychiatry 60:388–392

    Article  CAS  Google Scholar 

  32. Oh SJ, Dropcho EJ, Claussen GC (1997) Anti-Hu-associated paraneoplastic sensory neuropathy responding to early aggressive immunotherapy: report of two cases and review of literature. Muscle Nerve 20:1576–1582

    Article  CAS  Google Scholar 

  33. Keime-Guibert F, Graus F, Fleury A, René R, Honnorat J, Broet P et al (2000) Treatment of paraneoplastic neurological syndromes with antineuronal antibodies [anti-Hu, anti-Yo] with a combination of immunoglobulins, cyclophosphamide, and methylprednisolone. J Neurol Neurosurg Psychiatry 68:479–482

    Article  CAS  Google Scholar 

  34. Shams'ili S, de Beukelaar J, Gratama JW, Hooijkaas H, van den Bent M, van't Veer M et al (2006) An uncontrolled trial of rituximab for antibody associated paraneoplastic neurological syndromes. J Neurol 253:16–20

    Article  CAS  Google Scholar 

  35. Giometto B, Vitaliani R, Lindeck-Pozza E, Grisold W, Vedeler C (2012) Treatment for paraneoplastic neuropathies. Cochrane Database Syst Rev 12:CD007625

    PubMed  Google Scholar 

  36. Graus F, Delattre JY, Antoine JC, Dalmau J, Giometto B, Grisold W et al (2004) Recommended diagnostic criteria for paraneoplastic neurological syndromes. J Neurol Neurosurg Psychiatry 75:1135–1140

    Article  CAS  Google Scholar 

  37. Cheli M, Dinoto A, Ridolfi M, Sartori A, Stokelj D, Manganotti P (2019) Motor neuron disease as a treatment responsive paraneoplastic neurological syndrome in patient with small cell lung cancer, anti-Hu antibodies and limbic encephalitis. J Neurol Sci 15(400):158–159

    Article  Google Scholar 

  38. Goodfellow J, Gorrie G, Leach V, Patel S, Mackay G (2019) Cancer and motor neuron disease-causal or coincidental? Two contrasting cases. Neurol Sci 40:1461–1463

    Article  Google Scholar 

  39. Struck AF, Salamat S, Waclawik AJ (2014) Motor neuron disease with selective degeneration of anterior horn cells associated with non-Hodgkin lymphoma. J Clin Neuromuscul Dis 16:83–89

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Neurology Department, Okmeydani Training and Research Hospital, Istanbul, Turkey

    Onur Akan

  2. Neurology Department, Trakya University Hospital, Edirne, Turkey

    Leyla Baysal-Kirac

Authors
  1. Onur Akan
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Leyla Baysal-Kirac
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Leyla Baysal-Kirac.

Ethics declarations

Conflict of interest

No potential conflict of interest was reported by the authors.

Ethical approval

This article does not contain any studies with human participants or animals performed by any of the authors.

Informed consent

For this type of study formal consent is not required.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Akan, O., Baysal-Kirac, L. Amyotrophic lateral sclerosis with coexisting cancer: a single-center study. Acta Neurol Belg 121, 1123–1130 (2021). https://doi.org/10.1007/s13760-020-01337-y

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s13760-020-01337-y

Keywords

Search

Navigation