Endocrine neuroimmunology

Harry Alexopoulos DPhil (Dr. Alexopoulos of the National and Kapodistrian University of Athens has no relevant financial relationships to disclose.)
Marinos C Dalakas MD (Dr. Dalakas of the University of Athens Medical School and Thomas Jefferson University received honorariums from Baxter, Hoffmann LaRoche, Novartis, and Therapath for consulting, serving on advisory committees, and speaking engagements.)
Raymond P Roos MD, editor. (Dr. Roos of the University of Chicago owns stock in Amgen, Express Scripts, Isis, and Merck.)
Originally released July 9, 2001; last updated February 18, 2016; expires February 18, 2019

This article includes discussion of endocrine neuroimmunology, autoimmune thyroid disease associated with encephalopathy, glutamic acid decarboxylase autoantibody-related disorders with nervous system manifestations, Hashimoto encephalopathy, organ-specific antibodies, polyglandular autoimmune syndromes, polyglandular autoimmune syndromes with nervous system manifestations, type II autoimmune polyglandular syndrome, and type II autoimmune polyglandular syndrome. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Endocrine neuroimmunology refers to a group of clinically diverse neurologic disorders characterized by autoimmunity concurrently directed against endocrine and nervous system components. As a result, the patients present with clinical features related to involvement of an endocrine organ and the central or peripheral nervous system. Several endocrine disorders result from immune-mediated processes including type I diabetes mellitus, autoimmune thyroid disease, idiopathic Addison disease, and hypoparathyroidism. Involvement of the nervous system in these syndromes is usually secondary, caused by endocrine dysfunction (ie, peripheral neuropathy associated with diabetes). There are, however, neurologic conditions associated with autoimmune endocrine syndromes that are not causally related to endocrine complications, but to circulating autoantibodies or sensitized T cells that concurrently recognize common cellular antigens in both the endocrine and the nervous tissue.

In this article, most of the neurologic disorders associated with autoimmune endocrinopathies are reviewed. Among them, the most overlooked but potentially treatable is Hashimoto encephalopathy, which presents with subacute onset and rapidly progressive cognitive impairment, regardless of age. The disorder can be reversed after intravenous administration of steroids or other immunomodulatory therapy. Another member of this group of disorders is autoimmune thyroid ophthalmopathy or Graves ophthalmopathy, which is also a treatable condition if therapy is initiated early. The second group consists of the glutamic acid decarboxylase (GAD) autoantibody-related neurologic disorders, in which autoantibodies target pancreatic cells, resulting in diabetes, and the GABAergic pathways in the central nervous system, resulting in stiff-person syndrome and, less often, cerebellar ataxia, temporal lobe epilepsy, myoclonus, and nonparaneoplastic limbic encephalitis. Finally, POEMS syndrome includes a wide array of multiorgan and multiendocrine symptoms and signs, with sensorimotor peripheral neuropathy being the main neurologic manifestation.

Key points

 

• Endocrine neuroimmunology describes either endocrine syndromes with neurologic involvement or neurologic disorders with autoimmunity targeting antigens of the endocrine system.

 

• The most significant disorders or groups of disorders are Hashimoto encephalopathy, autoimmune thyroid ophthalmopathy, anti-GAD antibody-associated disorders, and POEMS syndrome.

 

• The precise pathogenetic mechanisms and the role of circulating antibodies in the aforementioned endocrine neuroimmunological disorders have not yet been elucidated. However, significant progress has been made in understanding the immunopathogenesis of anti-GAD antibody-mediated disorders and Graves ophthalmopathy.

 

• All of these disorders, although not very common in clinical practice, are important to diagnose early because they respond to immunomodulatory treatments to a varying degree and for a period of time.

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