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

Kyle M Blackburn; Maddie Kubiliun; Samar Harris; Steven Vernino

doi:10.1111/nmo.13611

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.

Authors

Kyle M Blackburn¹, Maddie Kubiliun², Samar Harris², Steven Vernino¹

Affiliations

¹ Department of Neurology and Neurotherapeutics, University of Texas Southwestern Medical Center, Dallas, Texas.
² Division of Gastroenterology and Hepatology, Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, Texas.

PMID: 31016817
DOI: 10.1111/nmo.13611

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