A Case of Syncope and Impaired Micturition
Panautonomic failure is manifested by a variety of symptoms indicating dysfunction of the sympathetic, parasympathetic, and enteric autonomic systems. An acute onset of this syndrome, in the absence of other systemic illness or neurological symptoms and signs, indicates autoimmune autonomic ganglionopathy (AAG), in which symptoms are causally related to the presence of antibodies to ganglionic nicotinic acetyl choline receptors. Differential diagnosis consists principally of toxic/metabolic disorders and other autoimmune illnesses, most notably Sjögren’s syndrome. Antibodies to ganglionic nicotinic acetyl choline receptors are present in 50–60% of cases, and, unlike in some other antibody-mediated disorders, symptom severity is related to the antibody level. Paroxysmal coughing spells are a feature of the illness in Japanese, but not Western, patients. Corticosteroids are generally ineffective when used alone, but may provide better response when used in combination with IVIg or plasma exchange. Other immunosuppressive medications are often required.