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Paraneoplastic Diseases of the Peripheral Nervous System

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Acquired Neuromuscular Disorders

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Abstract

Peripheral nervous system involvement is the most commonly observed condition in patients with paraneoplastic neurological syndromes (PNS), and its clinical manifestations are highly heterogeneous. The peripheral nervous system can be variously involved, but the most frequently affected sites are the dorsal ganglia and presynaptic nerve endings of the neuromuscular junction. While peripheral nervous system damage can be induced by various mechanisms in patients with systemic malignancy, in patients with paraneoplastic neuropathy, it is usually of autoimmune origin and only rarely associated with direct tumour infiltration or caused by chemotherapy. Metabolic-related causes are also uncommon. PNS arise in less than 1 % of patients with malignancy, preceding the diagnosis of cancer by months or even years in the majority of cases [43]. Specific serological markers can be used to screen for classical paraneoplastic syndromes, as defined by Graus et al. [22]. Subacute sensory neuropathy and Lambert-Eaton myasthenic syndrome (LEMS) are classified as classical syndromes. Onconeural antibodies directed against neural antigens expressed by the tumour may occur in most affected patients, suggesting an underlying autoimmune process [19].

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References

  1. Allen D, Lunn MP, Niermeijer J, Nobile-Orazio E (2007) Treatment for IgG and IgA paraproteinaemic neuropathy. Cochrane Database Syst Rev (1):CD005376

    Google Scholar 

  2. Antoine JC, Mosnier JF, Absi L, Convers P, Honnorat J, Michel D (1999) Carcinoma associated paraneoplastic peripheral neuropathies in patients with and without anti-onconeuronal antibodies. J Neurol Neurosurg Psychiatry 67:7–14

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  3. Argyriou AA, Kyritsis AP, Makatsoris T, Kalofonos HP (2014) Chemotherapy-induced peripheral neuropathy in adults: a comprehensive update of the literature. Cancer Manage Res 6:135–147

    Article  Google Scholar 

  4. Ayyappan S, Day T, Kiers L (2015) Distal acquired demyelinating symmetric (DADS) neuropathy associated with colorectal adenocarcinoma. Muscle Nerve 51(6):928–931

    Article  CAS  PubMed  Google Scholar 

  5. Bayat E, Kelly JJ (2012) Neurologic complications in plasma cell dyscrasias. In: Aminoff MJ, Boller F, Swaab DF (eds) Handbook of clinical neurology, 104 and 105, vol 3, Neurooncology, part 2. Elsevier, Edinburgh, pp 731–774

    Google Scholar 

  6. Briani C, Vitaliani R, Grisold W et al (2011) Spectrum of paraneoplastic disease associated with lymphoma. Neurology 76:705–710

    Article  CAS  PubMed  Google Scholar 

  7. Camdessanche JP, Antoine JC, Honnorat J, Vial C, Petiot P, Convers P, Michel D (2002) Paraneoplastic peripheral neuropathy associated with anti-Hu antibodies. A clinical and electrophysiological study of 20 patients. Brain 125:166–175

    Article  PubMed  Google Scholar 

  8. Clouston PD, Saper CB, Arbizu T, Jhonston I, Lang B, Posner JB, Newsom-Davis J (1992) Paraneoplastic cerebellar degeneration. III. Cerebellar degeneration, cancer, and the Lambert-Eaton myasthenic syndrome. Neurology 42:1944–1950

    Article  CAS  PubMed  Google Scholar 

  9. Dalmau J, Graus F, Rosenblum MK, Posner JB (1992) Anti-Hu associated paraneoplastic encephalomyelitis/sensory neuronopathy. A clinical study of 71 patients. Medicine (Baltimore) 71:59–72

    Article  CAS  Google Scholar 

  10. Denny-Brown D (1948) Primary sensory neuropathy with muscular changes associated with carcinoma. J Neurol Neurosurg Psychiatry 11:73–87

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  11. Dispenzieri A (2007) POEMS syndrome. Blood Rev 21:285–299

    Article  PubMed  Google Scholar 

  12. Drlicek M, Bodenteich A, Setinek U, Tucek G, Urbanits S, Grisold W (2000) T cell-mediated paraneoplastic ganglionitis: an autopsy case. Acta Neuropathol 99:599–602

    Article  CAS  PubMed  Google Scholar 

  13. Eggers C, Hagel C, Pfeiffer G (1998) Anti-Hu-associated paraneoplastic sensory neuropathy with peripheral nerve demyelination and microvasculitis. J Neurol Sci 155:178–181

    Article  CAS  PubMed  Google Scholar 

  14. Fonseca R, Hayman S (2007) Waldenström macroglobulinaemia. Br J Haematol 138:700–720

    Article  CAS  PubMed  Google Scholar 

  15. Gawler J (2004) Neurological manifestations of myeloma and their management. In: Malpas JS, Bergsagel DE, Kyl RA, Anderson KC (eds) Myeloma: biology and management, 3rd edn. Saunders, Elsevier Inc, Philadelphia, pp 269–293

    Google Scholar 

  16. Giometto B, Tavolato B, Graus F (1999) Autoimmunity in paraneoplastic neurological syndromes. Brain Pathol 9:261–274

    Article  CAS  PubMed  Google Scholar 

  17. Graus F, Dalmau J, Rene R, Tora M, Malats N, Verschuuren JJ, Cardenal F, Vinolas J, del Muro G, Vadell C, Mason WP, Rosell R, Posner JB, Real FX (1997) Anti-Hu antibodies in patients with small-cell lung cancer: association with complete response to therapy and improved survival. J Clin Oncol 51:2866–2872

    Google Scholar 

  18. Graus F, Keime-Guilbert F, Renè R, Benyahia B, Ribalta T, Ascaso C, Escaramis G, Delattre Y (2001) Anti-Hu associated paraneoplastic encephalomyelitis: analysis of 200 patients. Brain 124:1138–1148

    Article  CAS  PubMed  Google Scholar 

  19. Graus F, Delattre JY, Antoine JC, Dalmau J, Giometto B, Grisold W, Honnorat J, Smitt PS, Vedeler C, Verschuuren JJ, Vincent A, Voltz R (2004) Recommended diagnostic criteria for paraneoplastic neurological syndromes. J Neurol Neurosurg Psychiatry 78:1135–1140

    Article  Google Scholar 

  20. Grisariu S, Avni B, Batchelor TT et al (2010) Neurolymphomatosis: an International Primary CNS Lymphoma Collaborative Group report. Blood 115:5005–5011

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. Grisold W, Grisold A (2014) Cancer around the brain. Neurooncol Pract 1:13–21

    PubMed  PubMed Central  Google Scholar 

  22. Grisold W, Grisold A, Hainfellner J, Meng S, Marosi C (2014) Leukemia and the peripheral nervous system: a review. J Leuk (Los Angel) 2:162. doi:10.4172/2329-6917.1000162

    Google Scholar 

  23. Grisold W, Klimpfinger M, Maehr B et al (2007) Peripheral nerve involvement in lymphoma: the meninges as the crucial barrier between meningoradicular spread and neurolymphomatosis. J Peripher Nerv Syst 12:58–60

    Article  PubMed  Google Scholar 

  24. Hadden RD, Nobile-Orazio E, Sommer C, Hahn A, Illa I, Morra E, Pollard J, Hughes RA, Bouche P, Cornblath D, Evers E, Koski CL, Léger JM, Van den Bergh P, van Doorn P, van Schaik IN (2006) European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of paraproteinaemic demyelinating neuropathies: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society. Eur J Neurol 13:809–818

    Article  CAS  PubMed  Google Scholar 

  25. Hart IK, Maddison P, Newson-DavisJ VA, Mills KR (2002) Phenotypic variants of autoimmune peripheral nerve hyperexcitability. Brain 125:1887–1895

    Article  PubMed  Google Scholar 

  26. Isaacs H (1961) A syndrome of continuous muscle fibre activity. J Neurol Neurosurg Psychiatry 24:319–325

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  27. Johnson PC, Rolak LA, Hamilton RH, Laguna JF (1979) Paraneoplastic vasculitis of nerve: a remote effect of cancer. Ann Neurol 5:437–444

    Article  CAS  PubMed  Google Scholar 

  28. Kannan MA, Challa S, Kandadai RM, Uppin MS, Jabeen SA, Borgohain R (2015) Series of paraneoplastic vasculitic neuropathy: a rare, potentially treatable neuropathy. Neurol India 63:30–34

    Article  PubMed  Google Scholar 

  29. Latov N, Hays AP, Sherman WH (1988) Peripheral neuropathy and anti-MAG antibodies. Crit Rev Neurobiol 3:301–332

    CAS  PubMed  Google Scholar 

  30. Levine T, Pestronk A, Florence J, Al-Lozi MT, Lopate G, Miller T, Ramneantu I, Waheed W, Stambuk M, Stone MJ, Choksi R (2006) Peripheral neuropathies in Waldenström’s macroglobulinaemia. J Neurol Neurosurg Psychiatry 77:224–228

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  31. Lorusso L, Hart IK, Ferrari D, Ngonga GK, Gasparetto C, Ricevuti G (2007) Autonomic paraneoplastic neurological syndromes. Autoimmun Rev 6:162–168

    Article  CAS  PubMed  Google Scholar 

  32. Lozeron P, Adams D (2007) Monoclonal gammopathy and neuropathy. Curr Opin Neurol 20:536–541

    CAS  PubMed  Google Scholar 

  33. Lunn MP, Nobile-Orazio E (2006) Immunotherapy for IgM anti-myelin-associated glycoprotein paraprotein-associated peripheral neuropathies. Cochrane Database Syst Rev (2):CD002827

    Google Scholar 

  34. Mols F, Beijers T, Vreugdenhil G, van de Poll-Franse L, information A (2014) Chemotherapy-induced peripheral neuropathy and its association with quality of life: a systematic review. Support Care Cancer 22(8):2261–2269

    Article  PubMed  Google Scholar 

  35. Oh SJ (1997) Paraneoplastic vasculitis of the peripheral nervous system. Neurol Clin 15:849–863

    Article  CAS  PubMed  Google Scholar 

  36. Oh SJ, Gurtekin Y, Dropcho EJ, King P, Claussen GC (2005) Anti-Hu antibody neuropathy: a clinical, electrophysiological, and pathological study. Clin Neurophysiol 116:28–34

    Article  PubMed  Google Scholar 

  37. O’Neill J, Murray NM, Nesom-Davis J (1988) Lambert and Eaton myasthenic syndrome: a review of 50 cases. Brain 111:577–596

    Article  PubMed  Google Scholar 

  38. Posner JB (1995) Paraneoplastic syndromes. In: Posner JB (ed) Neurologic complication of cancer, Contemporary neurology series. F.A. Davis Company, Philadelphia, pp 353–383

    Google Scholar 

  39. Reddy CG, Mauermann ML, Solomon BM et al (2012) Neuroleukemiosis: an unusual cause of peripheral neuropathy. Leuk Lymphoma 53:2405–2411

    Article  PubMed  Google Scholar 

  40. Velasco R, Bruna J, Briani C et al (2014) Early predictors of oxaliplatin-induced cumulative neuropathy in colorectal cancer patients. J Neurol Neurosurg Psychiatry 85(4):392–398. doi:10.1136/jnnp-2013-305334

    Article  PubMed  Google Scholar 

  41. Scarlato M, Previtali SC, Carpo M, Pareyson D, Briani C, Del Bo R, Nobile-Orazio E, Quattrini A, Comi GP (2005) Polyneuropathy in POEMS syndrome: role of angiogenic factors in the pathogenesis. Brain 128:1911–1920

    Article  PubMed  Google Scholar 

  42. Tomita M, Koike H, Kawagashira Y et al (2013) Clinicopathological features of neuropathy associated with lymphoma. Brain J Neurol 136:2563–2578

    Article  Google Scholar 

  43. Verstappen CC et al (2003) Neurotoxic complications of chemotherapy in patients with cancer: clinical signs and optimal management. Drugs 63:1549–1563

    Article  CAS  PubMed  Google Scholar 

  44. Vigliani MC, Magistrello M, Polo P, Mutani R, Chiò A; Piemonte and Valle d’Aosta Register for Guillain-Barré Syndrome (2004). Risk of cancer in patients with Guillain-Barré syndrome (GBS). A population-based study. J Neurol 251:321–326

    Google Scholar 

  45. Visser NA, Notermans NC, Linssen RS, van den Berg LH, Vrancken AF (2015) Incidence of polyneuropathy in Utrecht, the Netherlands. Neurology 84:259–264

    Article  CAS  PubMed  Google Scholar 

  46. Younger DS, Dalmau J, Inghirami G, Sherman WH, Hays AP (1994) Anti-Hu-associated peripheral nerve and muscle microvasculitis. Neurology 44(1):181–183

    Article  CAS  PubMed  Google Scholar 

  47. Wakerley BR, Uncini A, Yuki N, Group GBSC, Group GBSC (2014) Guillain-Barre and Miller Fisher syndromes–new diagnostic classification. Nat Rev Neurol 10:537–544

    Article  PubMed  Google Scholar 

  48. Watanabe O, Arimura K, Kitajima I, Osame M, Maruyama I (1996) Greatly raised vascular endothelial growth factor (VEGF) in POEMS syndrome. Lancet 347:702

    Article  CAS  PubMed  Google Scholar 

  49. Wirtz PW, Smallegange TM, Wintzen AR, Verschuuren JJ (2002) Differences in clinical features between the Lambert-Eaton myasthenic syndrome with and without cancer: and analysis of 227 published cases. Clin Neurol Neurosurg 104:359–363

    Article  PubMed  Google Scholar 

  50. Wirtz PW, Wintzen AR, Verschuuren JJ (2005) Lambert-Eaton myasthenic syndrome has a more progressive course in patients with lung cancer. Muscle Nerve 32:226–229

    Article  PubMed  Google Scholar 

  51. Zivkovic SA, Ascherman D, Lacomis D (2007) Vasculitic neuropathy–electrodiagnostic findings and association with malignancies. Acta Neurol Scand 115(6):432–436

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Neurology, S Antonio Hospital, Azienda ULSS 16, Padova, 35128, Italy

    Bruno Giometto MD

  2. Department of Neurology, University Clinic of Neurology, Vienna, Austria

    Anna Grisold MD

  3. Department of Neurology, Kaiser Franz Josef Hospital, Vienna, Austria

    Wolfgang Grisold MD PhD

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  1. Bruno Giometto MD
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  2. Anna Grisold MD
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Correspondence to Bruno Giometto MD .

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Editors and Affiliations

  1. Neuromuscular Center, IRCCS Fondazione San Camillo Hospit, Venice, Italy

    Corrado Angelini

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Giometto, B., Grisold, A., Grisold, W. (2016). Paraneoplastic Diseases of the Peripheral Nervous System. In: Angelini, C. (eds) Acquired Neuromuscular Disorders. Springer, Cham. https://doi.org/10.1007/978-3-319-29514-5_17

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  • DOI: https://doi.org/10.1007/978-3-319-29514-5_17

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