%0 Journal Article
%T Autonomic Involvement in Subacute and Chronic Immune-Mediated Neuropathies
%A Anna Mazzeo
%A Claudia Stancanelli
%A Rita Di Leo
%A Giuseppe Vita
%J Autoimmune Diseases
%D 2013
%I Hindawi Publishing Corporation
%R 10.1155/2013/549465
%X Autonomic function can be impaired in many disorders in which sympathetic, parasympathetic, and enteric arms of the autonomic nervous system are affected. Signs and symptoms of autonomic involvement are related to impairment of cardiovascular, gastrointestinal, urogenital, thermoregulatory, sudomotor, and pupillomotor autonomic functions. Availability of noninvasive, sensitive, and reproducible tests can help to recognize these disorders and to better understand specific mechanisms of some, potentially treatable, immune-mediated autonomic neuropathies. This paper describes autonomic involvement in immune-mediated neuropathies with a subacute or chronic course. 1. Introduction Among the peripheral neuropathies, there is a group in which autonomic fibers are prominently or selectively targeted. Disorders of autonomic function are present when small, lightly myelinated and unmyelinated nerve fibers are affected. Signs and symptoms of autonomic involvement are related to impairment of cardiovascular, gastrointestinal, urogenital, thermoregulatory, sudomotor, and pupillomotor autonomic functions. Autonomic dysfunction may occur in association with various diseases such as diabetes mellitus, chronic alcoholism, amyloidosis, infections, paraneoplastic syndrome, and Sjogren syndrome (secondary autonomic neuropathies) or without any underlying disease (idiopathic or primary autonomic neuropathies) [1]. Sensitive and reproducible noninvasive measures of autonomic function together with laboratory and electrophysiological testing can help to establish diagnosis, allowing to recognize potentially treatable immune-mediated disorders. Tests of cardiovagal (parasympathetic), adrenergic vasoconstriction (sympathetic) are now available in most laboratories and commonly performed [2]. This paper will focus on autonomic involvement in immune-mediated neuropathies with a subacute or chronic course. 2. Subacute and Chronic Neuropathies with Immune-Mediated Mechanisms and Autonomic Involvement 2.1. Immune-Mediated Idiopathic (Primary) Autonomic Neuropathies 2.1.1. Autoimmune Autonomic Ganglionopathy (AAG) AAG includes a wide spectrum of acquired disorders characterized by diffuse autonomic dysfunction with an immune-mediated pathophysiology and positivity of ganglionic nicotinic ¦Á3-acetylcholine receptors (¦Á3-AChR) autoantibodies. Viral upper respiratory tract infections and gastrointestinal infections have been described as antecedent infections. AAG may also be associated with vaccination, surgery, or interferon therapy. Classical AAG is a subacute disorder with a
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