Ocular manifestations of acute pandysautonomia.
A 33-year-old white man developed premature presbyopia and anisocoria as initial manifestations of acute pandysautonomia. Nine months later signs and symptoms of generalized, severe autonomic dysfunction developed, and six years later only paralysis of pupillary reactions, presbyopia, and orthostatic hypotension were unresolved. Pharmacologic testing of the pupils demonstrated no mydriasis to cocaine 4% or hydroxyamphetamine 1% and hypersensitivity to epinephrine 0.1%, methacholine 2.5%, and pilocarpine 0.0625%, suggesting the presence of sympathetic and parasympathetic, postganglionic blockage of autonomic innervation of the iris.
D000328:Adult; D001341:Autonomic Nervous System; D003250:Constriction; D004106:Dilatation; D004402:Dysautonomia, Familial / Q000175:diagnosis; D005128:Eye Diseases / Q000175:diagnosis*; D005260:Female; D006801:Humans; D007024:Hypotension, Orthostatic / Q000150:complications; D007037:Hypothyroidism / Q000150:complications; D008297:Male; D009422:Nervous System Diseases / Q000175:diagnosis; D011680:Pupil / Q000187:drug effects / Q000503:physiopathology; D014603:Uveal Diseases / Q000175:diagnosis