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A 66-year-old woman presented with asymptomatic tonic pupils and areflexia when she was admitted to our hospital due to vertigo. She had also noticed heat intolerance and decreased sweating on her right side since she was approximately 10 years old. Both sides of each eye contracted in the pilocarpine eye drop test, while sweating on the face and left chest decreased. She was thus diagnosed with Ross syndrome. When acethylcholine was administered intracutaneously, sweating of the left subclavian and left peri-umbilical areas decreased. During an electrogastrogram, the average muscle contraction frequency was decreased and the incidence of bradygastria increased. Blood flow of the skin of the left forefinger (but not right) increased at rest and the reaction in the blood flow on deep breathing decreased. A skin biopsy of the left upper arm showed atrophy of pilosebaceous and sweat glands. Despite treatment with intravenous immunoglobulin, the patient's deficits did not improve. The lesions typical of Ross syndrome may be associated with many parts of the autonomic nervous system.

Ross syndrome presenting with asymptomatic tonic pupils