Neurological autoimmune disorders with prominent gastrointestinal manifestations: A review of presentation, evaluation, and treatment

Neurogastroenterol Motil. 2019 Oct;31(10):e13611. doi: 10.1111/nmo.13611. Epub 2019 Apr 23.

Abstract

Background: The identification of autoantibodies directed against neuronal antigens has led to the recognition of a wide spectrum of neurological autoimmune disorders (NAD). With timely recognition and treatment, many patients with NAD see rapid improvement. Symptoms associated with NAD can be diverse and are determined by the regions of the nervous system affected. In addition to neurological symptoms, a number of these disorders present with prominent gastrointestinal (GI) manifestations such as nausea, diarrhea, weight loss, and gastroparesis prompting an initial evaluation by gastroenterologists.

Purpose: This review provides a general overview of autoantibodies within the nervous system, focusing on three scenarios in which nervous system autoimmunity may initially present with gut symptoms. A general approach to evaluation and treatment, including antibody testing, will be reviewed.

Keywords: autoimmune disease; autonomic neuropathy; chronic nausea and vomiting; paraneoplastic.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Aged
  • Antibodies, Antinuclear / immunology
  • Antibodies, Neoplasm
  • Aquaporin 4 / immunology
  • Area Postrema / physiopathology
  • Autoantibodies / immunology
  • Autoimmune Diseases of the Nervous System / complications
  • Autoimmune Diseases of the Nervous System / drug therapy
  • Autoimmune Diseases of the Nervous System / immunology
  • Autoimmune Diseases of the Nervous System / physiopathology*
  • Autonomic Nervous System Diseases / complications
  • Autonomic Nervous System Diseases / drug therapy
  • Autonomic Nervous System Diseases / immunology
  • Autonomic Nervous System Diseases / physiopathology
  • Brain / diagnostic imaging
  • Diarrhea / etiology
  • Diarrhea / immunology
  • Diarrhea / physiopathology
  • Dipeptidyl-Peptidases and Tripeptidyl-Peptidases / immunology
  • Female
  • Gastrointestinal Diseases / etiology
  • Gastrointestinal Diseases / immunology
  • Gastrointestinal Diseases / physiopathology*
  • Gastroparesis / etiology
  • Gastroparesis / immunology
  • Gastroparesis / physiopathology
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Intestinal Pseudo-Obstruction / complications
  • Intestinal Pseudo-Obstruction / drug therapy
  • Intestinal Pseudo-Obstruction / immunology
  • Intestinal Pseudo-Obstruction / physiopathology
  • Male
  • Middle Aged
  • Nausea / etiology
  • Nausea / immunology
  • Nausea / physiopathology
  • Nerve Tissue Proteins / immunology
  • Neuromyelitis Optica / complications
  • Neuromyelitis Optica / drug therapy
  • Neuromyelitis Optica / immunology
  • Neuromyelitis Optica / physiopathology
  • Paraneoplastic Syndromes, Nervous System / complications
  • Paraneoplastic Syndromes, Nervous System / drug therapy
  • Paraneoplastic Syndromes, Nervous System / immunology
  • Paraneoplastic Syndromes, Nervous System / physiopathology
  • Potassium Channels / immunology
  • Weight Loss

Substances

  • ANNA1 antibody
  • AQP4 protein, human
  • Antibodies, Antinuclear
  • Antibodies, Neoplasm
  • Aquaporin 4
  • Autoantibodies
  • Immunosuppressive Agents
  • Nerve Tissue Proteins
  • Potassium Channels
  • DPP6 protein, human
  • Dipeptidyl-Peptidases and Tripeptidyl-Peptidases