Skip to main content

Advertisement

Log in

Achalasia, chronic sensory neuropathy, and N-type calcium channel autoantibodies: beneficial response to IVIG

  • Case Report
  • Published:
Clinical Journal of Gastroenterology Aims and scope Submit manuscript

Abstract

Autoimmune gastrointestinal dysmotility (AGID) can result from paraneoplastic onconeuronal antibodies. Patients may present with regional hypomotility anywhere along the gastrointestinal tract. We report a case of a woman who developed an insidious sensory neuropathy and achalasia. She was found to have a high-titer of N-type voltage gated-calcium channel (VGCC) antibodies. She demonstrated clinical and electrophysiological improvement of her neuropathy, as well as improvement of her swallowing and gait, after treatment with intravenous immunoglobulins.

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

Access this article

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

Instant access to the full article PDF.

Fig. 1

Similar content being viewed by others

References

  1. Kraichely RE, Farrugia G. Achalasia: physiology and etiopathogenesis. Dis Esophagus. 2006;19:213–23.

    Article  CAS  PubMed  Google Scholar 

  2. Holloway RH, Dodds WJ, Helm JF, Hogan WJ, Dent J, Arndorfer RC. Integrity of cholinergic innervation to the lower esophageal sphincter in achalasia. Gastroenterology. 1986;90:924–9.

    CAS  PubMed  Google Scholar 

  3. Lennon VA, Sas DF, Busk MF, Scheithauer B, Malagelada JR, Camilleri M, et al. Enteric neuronal autoantibody in pseudoobstruction with small-cell lung carcinoma. Gastroenterology. 1991;100:137–42.

    CAS  PubMed  Google Scholar 

  4. Lucchinetti CF, Kimmel DW, Lennon VA. Paraneoplastic and oncologic profiles of patients seropositive for type 1 antineuronal nuclear autoantibodies. Neurology. 1998;50:652–7.

    CAS  PubMed  Google Scholar 

  5. Graus F, Keime-Guibert F, Reñe R, Benyahia B, Ribalta T, Ascaso C, et al. Anti-Hu associated paraneoplastic encephalomyelitis: analysis of 200 patients. Brain. 2001;124:1138–48.

    Article  CAS  PubMed  Google Scholar 

  6. Lee HR, Lennon VA, Camilleri M, Prather CM. Paraneoplastic gastrointestinal motor dysfunction: clinical and laboratory characteristics. Am J Gastroenterol. 2001;96(2):373–9.

    Article  CAS  PubMed  Google Scholar 

  7. Dhamija R, Tan KM, Pittock SJ, Foxx-Orenstein A, Benarroch E, Lennon VA. Serologic profiles aiding the diagnosis of autoimmune gastrointestinal dysmotility. Clin Gastroenterol Hepatol. 2008;6:988–92.

    Article  PubMed  Google Scholar 

  8. Knowles CH, Lang B, Clover L, Scott SM, Gotti C, Vincent A, et al. A Role for autoantibodies in some cases of acquired non-paraneoplastic gut dysmotility. Scand J Gastroenterol. 2002;37:166–70.

    Article  CAS  PubMed  Google Scholar 

  9. Tornblum H, Lang B, Clover L, Knowles CH, Vincent A, Lindberg G. Autoantibodies in patients with gut motility disorders and enteric neuropathy. Scand J Gastroenterol. 2007;42:1289–93.

    Article  Google Scholar 

  10. Vernino S, Low PA, Fealey RD, Steward JD, Farrugia G, Lennon VA. Autoantibodies to ganglionic acetylcholine receptors in autoimmune autonomic neuropathies. N Engl J Med. 2000;343:847–55.

    Article  CAS  PubMed  Google Scholar 

  11. Waterman SA. Voltage-gated calcium channels in autonomic neuroeffector transmission. Prog Neurobiol. 2000;60:181–210.

    Article  CAS  PubMed  Google Scholar 

  12. Malmberg AB, Yaksh TL. Effect of continuous intrathecal infusion of omega-conopeptides, N-type calcium-channel blockers, on behavior and antinociception in the formalin and hot-plate tests in rats. Pain. 1995;60:83–90.

    Article  CAS  PubMed  Google Scholar 

  13. Lennon V, Kryzer TJ, Griesmann GE, O’Suilleabhain PE, Windebank AJ, Woppman A, et al. Calcium-channel antibodies in the Lambert-Eaton syndrome and other paraneoplastic syndromes. New Engl J Med. 1995;332:1467–74.

    Article  CAS  PubMed  Google Scholar 

  14. Chalk CH, Windebank AJ, Kimmel DW, McManis PG. The distinctive clinical features of paraneoplastic sensory neuronopathy. Can J Neurol Sci. 1992;19:346–51.

    CAS  PubMed  Google Scholar 

  15. Mori K, Iijima M, Koike H, Hattori N, Tanaka F, Watanabe H, et al. The wide spectrum of clinical manifestations in Sjogren’s syndrome-associated neuropathy. Brain. 2005;128:2518–34.

    Article  PubMed  Google Scholar 

  16. Morozumi S, Kawagashira Y, Iijima M, Koike H, Hattori N, Katsuno M, et al. Intravenous immunoglobulin treatment for painful sensory neuropathy associated with Sjögren’s syndrome. J Neurol Sci. 2009;279:57–61.

    Article  CAS  PubMed  Google Scholar 

  17. Takahashi Y, Takata T, Hoshino M, Sakurai M, Kanazawa I. Benefit of IVIG for long-standing ataxic sensory neuronopathy with Sjögren’s syndrome. Neurology. 2003;60:503–5.

    CAS  PubMed  Google Scholar 

  18. Burns TM, Quijano-Roy S, Jones HR, Wolfe GI, Nations SP, Burns DK, et al. Benefit of IVIG for long-standing ataxic sensory neuronopathy with Sjogren’s syndrome [Letter]. Neurology. 2003;61:873.

    Google Scholar 

  19. Chen WH, Yeh JH, Chiu HC. Plasmapheresis in the treatment of ataxic sensory neuropathy associated with Sjogren’s syndrome. Eur Neurol. 2001;45:270–4.

    Article  PubMed  Google Scholar 

  20. Widdess-Walsh P, Tavee JO, Schuele S, Stevens GH. Response to intravenous immunoglobulin in anti-Yo associated paraneoplastic cerebellar degeneration: case report and review of the literature. J Neurooncol. 2003;63:187–90.

    Article  PubMed  Google Scholar 

  21. Phuphanich S, Brock C. Neurologic improvement after high-dose intravenous immunoglobulin therapy in patients with paraneoplastic cerebellar degeneration associated with anti-Purkinje cell antibody. J Neurooncol. 2007;81:67–9.

    Article  CAS  PubMed  Google Scholar 

  22. Keime-Guibert F, Graus F, Fleury A, Rene R, Honnorat J, Broet P, et al. Treatment of paraneoplastic neurological syndromes with antineuronal antibodies (anti-Hu, anti-Yo) with a combination of immunoglobulins, cyclophosphamide and methylprednisolone. J Neurol Neurosurg Psychiatry. 2000;68:479–82.

    Article  CAS  PubMed  Google Scholar 

  23. Schäppi MG, Smith VV, Milla PJ, Lindley KJ. Eosinophilic myenteric ganglionitis is associated with functional intestinal obstruction. Gut. 2003;52:752–5.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Jayashri Srinivasan.

Rights and permissions

Reprints and permissions

About this article

Cite this article

McMillan, H.J., Srinivasan, J. Achalasia, chronic sensory neuropathy, and N-type calcium channel autoantibodies: beneficial response to IVIG. Clin J Gastroenterol 3, 78–82 (2010). https://doi.org/10.1007/s12328-010-0140-6

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12328-010-0140-6

Keywords

Navigation