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

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...



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 ( 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.


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Copyright information

© Fondazione Società Italiana di Neurologia 2019

Authors and Affiliations

  1. 1.Department of NeurologyAomori Prefectural Central HospitalAomoriJapan

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