Advertisement

Autoimmune autonomic ganglionopathy associated with monoclonal gammopathy of undetermined significance: a case report

  • Tatsuya UenoEmail author
  • Takashi Nakamura
  • Akira Arai
  • Chieko Suzuki
  • Masahiko Tomiyama
Letter to the Editor
  • 16 Downloads

Dear Editor:

Autoimmune autonomic ganglionopathy (AAG) is an acquired immune-mediated disorder characterized by severe autonomic failure [1]. Anti-ganglionic nicotinic acetylcholine receptor (gAchR) antibody is detected in approximately 50% of cases of acute or subacute AAG [1]. About 15% of AAG cases are also complicated by cancer, e.g., small cell lung carcinoma [1]. Previous reports suggested an association of multiple myeloma (MM) with AAG [2, 3]; however, no reports suggest an association between AAG and monoclonal gammopathy of undetermined significance (MGUS). The overproduction of monoclonal light chains causes amyloid light chain (AL) amyloidosis leading to autonomic failure; therefore, it is important to distinguish AAG from AL amyloidosis. We, herein, report a patient who had AAG and MGUS without deposition.

A 70-year-old Japanese man who suffered from syncope due to orthostatic hypotension was admitted to our department. He had constipation at 65 years old, dysuria at...

Notes

Acknowledgements

The authors would like to thank Dr. Tomoaki Akagi, Department of Hematology, Aomori Prefectural Central Hospital, Aomori, Japan, for performing the hematological evaluation. We thank S. Win, PhD, from Edanz Group (www.edanzediting.com/ac) for editing a draft of this manuscript.

Compliance with ethical standards

Consent for publication

Written informed consent was obtained from the patient for publication of this case report and any accompanying images.

References

  1. 1.
    Vernino S (2007) Autoimmune and paraneoplastic channelopathies. Neurotherapeutics 4(2):305–314.  https://doi.org/10.1016/j.nurt.2007.01.007 CrossRefPubMedGoogle Scholar
  2. 2.
    McKeon A, Lennon VA, Lachance DH, Fealey RD, Pittock SJ (2009) Ganglionic acetylcholine receptor autoantibody: oncological, neurological, and serological accompaniments. Arch Neurol 66(6):735–741.  https://doi.org/10.1001/archneurol.2009.78 CrossRefPubMedPubMedCentralGoogle Scholar
  3. 3.
    Nakae Y, Hyuga M, Terada Y, Kishimoto W, Fukunaga A, Tabata S, Maesako Y, Komatsu K, Higuchi O, Nakane T, Arima N (2017) Multiple myeloma presenting with autoimmune autonomic ganglionopathy. Intern Med 56(24):3347–3351.  https://doi.org/10.2169/internalmedicine.9096-17 CrossRefPubMedPubMedCentralGoogle Scholar
  4. 4.
    Lindqvist EK, Goldin LR, Landgren O, Blimark C, Mellqvist UH, Turesson I, Wahlin A, Bjorkholm M, Kristinsson SY (2011) Personal and family history of immune-related conditions increase the risk of plasma cell disorders: a population-based study. Blood 118(24):6284–6291.  https://doi.org/10.1182/blood-2011-04-347559 CrossRefPubMedPubMedCentralGoogle Scholar
  5. 5.
    Sugiyama A, Asahina M, Takeda Y, Shiojiri T, Sano K, Ikeda S, Kuwabara S (2014) Isolated autonomic failure without evident somatic polyneuropathy in AL amyloidosis. Amyloid 21(3):218–220.  https://doi.org/10.3109/13506129.2014.935321 CrossRefPubMedGoogle Scholar

Copyright information

© Fondazione Società Italiana di Neurologia 2019

Authors and Affiliations

  1. 1.Department of NeurologyAomori Prefectural Central HospitalAomoriJapan

Personalised recommendations