Seronegative and seropositive autoimmune autonomic ganglionopathy (AAG): Same clinical picture, same response to immunotherapy

J Neuroimmunol. 2018 Jun 15:319:68-70. doi: 10.1016/j.jneuroim.2018.03.018. Epub 2018 Apr 4.

Abstract

Two patients with a syndrome of pandisautonomia with clinical criteria of AAG are provided. Both patients present a similar clinical picture and response to immunosuppressive treatment. One of them has positive antibodies against the ganglionic nicotinic acetylcholine (gAChr) and the other does not. This brief article serves to reflect the spectrum of AAG, at a clinical level, in laboratory tests and in the response to immunotherapy, independently of the presence of positive gAChr antibodies.

Keywords: Autoimmune autonomic ganglionopathy (AAG); Autonomic failure; Ganglionic nicotinic acetylcholine receptor antibody (gAChr); Postural orthostatic tachycardia syndrome (POTS).

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Autoantibodies / immunology
  • Autoantigens / immunology
  • Autoimmune Diseases of the Nervous System / drug therapy*
  • Autoimmune Diseases of the Nervous System / immunology*
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use*
  • Male
  • Middle Aged
  • Primary Dysautonomias / drug therapy*
  • Primary Dysautonomias / immunology*

Substances

  • Autoantibodies
  • Autoantigens
  • Immunoglobulins, Intravenous