[A case of autoimmune autonomic neuropathy with marked orthostatic hypotension, decreased salivation, constipation, and Adie pupil].

We report a 68-year-old man with subacute autonomic failure who developed orthostatic hypotension, decreased salivation, constipation, and Adie pupil. There was no evidence of central neurodegeneration, sensory and motor dysfunction. Head-up tilt test showed marked orthostatic hypotension without tachycardia. The patient had a low plasma noradrenaline concentration while supine and a poor noradrenaline response to head-up. Adie pupil responded to 0.125% pilocarpine, establishing denervation hypersensitivity of the parasympathetic nerve. Blood pressure, However, did not show a remarkable response to 0.05-0.2gamma noradrenaline, demonstrating the absence of denervation hypersensitivity of the sympathetic nerve. In contrast to patients with pure autonomic failure, the patient showed normal uptake of I-123-MIBG in the myocardium, indicating intact postganglionic sympathetic innervation. Serologic testing demonstrated circulating antibody to the ganglionic acetylcholine receptor (AchR). Because of subacute clinical course, autonomic symptoms and presence of anti-ganglionic AchR antibody, autoimmune autonomic neuropathy was diagnosed.