Case of autoimmune autonomic ganglionopathy manifesting anhidrosis

J Dermatol. 2017 Oct;44(10):1160-1163. doi: 10.1111/1346-8138.13870. Epub 2017 Jun 16.

Abstract

Autoimmune autonomic ganglionopathy (AAG), clinically characterized by gastrointestinal dysmotility, orthostatic hypotension and tonic pupils, is an idiopathic acquired disorder of the autonomic nervous system elicited by antibodies against ganglionic acetylcholine receptor (gAChR). We encountered a 60-year-old man who presented with severe anhidrosis, difficulty in thermoregulation, orthostatic hypotension, gastrointestinal dysmotility, tonic pupils and ptosis. Histologically, an anhidrotic skin sample was normal. Routine laboratory examinations of blood, urine and cerebrospinal fluid returned no abnormal findings. Serological examination revealed antibodies against α3 and β4 subunits of gAChR. The diagnosis was AAG. As sudomotor dysfunction reflects ganglionic neuropathy in AAG, we concluded that his anhidrosis was attributable to AAG. Anhidrosis is an important clue for the diagnosis of AAG, a rare neurological disorder.

Keywords: Horner's syndrome; anhidrosis; autoimmune autonomic ganglionopathy; autoimmune disease; ganglionic acetylcholine receptor.

Publication types

  • Case Reports

MeSH terms

  • Autoantibodies / blood*
  • Autoantibodies / immunology
  • Autoimmune Diseases of the Nervous System / blood
  • Autoimmune Diseases of the Nervous System / complications*
  • Autoimmune Diseases of the Nervous System / immunology
  • Autoimmune Diseases of the Nervous System / therapy
  • Blepharoptosis / blood
  • Blepharoptosis / etiology
  • Blepharoptosis / therapy
  • Ganglia, Autonomic / drug effects
  • Ganglia, Autonomic / immunology*
  • Ganglia, Autonomic / pathology
  • Gastrointestinal Diseases / blood
  • Gastrointestinal Diseases / etiology
  • Gastrointestinal Diseases / therapy
  • Gastrointestinal Motility / drug effects
  • Glucocorticoids / therapeutic use
  • Humans
  • Hypohidrosis / blood
  • Hypohidrosis / etiology*
  • Hypohidrosis / therapy
  • Hypotension, Orthostatic / blood
  • Hypotension, Orthostatic / etiology
  • Hypotension, Orthostatic / therapy
  • Immunoglobulins, Intravenous / therapeutic use
  • Male
  • Middle Aged
  • Nerve Tissue Proteins / immunology*
  • Plasmapheresis
  • Prednisolone / therapeutic use
  • Receptors, Nicotinic / immunology*
  • Skin / innervation
  • Skin / pathology
  • Treatment Failure

Substances

  • Autoantibodies
  • CHRNB4 protein, human
  • Glucocorticoids
  • Immunoglobulins, Intravenous
  • Nerve Tissue Proteins
  • Receptors, Nicotinic
  • nicotinic receptor subunit alpha3
  • Prednisolone