Chronic autoimmune autonomic neuropathy responsive to immunosuppressive therapy

Autoimmune autonomic neuropathy refers to a group of autoimmune disorders characterized by the failure of both sympathetic and parasympathetic systems,1 related to the presence of autoantibodies against neuronal ganglionic acetylcholine (AChR) receptors.2 These antibodies are detectable in the serum of about 30% of patients with paraneoplastic-acquired autoimmune autonomic neuropathy and in 50% of patients with idiopathic subacute autoimmune autonomic neuropathy.3 We describe a 59-year-old woman who presented 6 months after the gradual development of severe dysautonomia, characterized by recurrent orthostatic syncope, dry mouth, early satiety, constipation, and urinary retention. Family history was negative and she denied any viral illness preceding the onset of the above symptoms. The results of physical examination showed her blood pressure to be 115/70 mm Hg in a supine position, but fell to 80/50 mm Hg in a sitting position; the patient was unable to stand upright for more than a few seconds because of a fainting sensation. Pupillary responses to light were torpid; however, the remaining neurologic examination was normal. Routine blood tests, including glucose determination, were normal. X-ray gastrointestinal transit evaluation after barium ingestion documented markedly delayed gut motility …