Abstract
Ross syndrome is defined as the triad of tonic pupils, hyporeflexia, and segmental anhidrosis. It is an uncommon disorder and the nosologic distinction among Ross syndrome, Holmes-Adie syndrome (tonic pupil and areflexia), and harlequin syndrome (segmental anhidrosis without ocular sympathetic deficit) has been questioned. The authors describe the clinical findings of five patients with Ross syndrome and detail the results of their ocular pharmacological testing and additional autonomic testing. In four patients, the classic findings of Ross syndrome were accompanied by Horner’s syndrome, localizing to the third order (postganglionic) sympathetic neurons. Other symptoms and signs of dysautonomia were also common. These findings suggest that Ross syndrome is a dysautonomic condition of varying expression resulting from a generalized injury to the peripheral autonomic and dorsal root ganglia or their projections.—Nancy J. Newman
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