Anhidrosis: A clue to an underlying autonomic disorder

Abstract We describe a patient with Ross syndrome who had the classic triad of segmental anhidrosis, tonic pupils and hyporeflexia. Dilute pilocarpine (0.1%) instillation in both eyes revealed parasympathetic denervation hypersensitivity. There was evidence of cardiac dysautonomia in the form of a decrease in heart rate variability with deep breathing and an abnormal Valsalva response. A thermoregulatory sweat test showed the presence of sweating in the right cheek, neck and upper one-third of the left arm and shoulder only. Sympathetic skin responses were absent in the affected segments of the upper limb. Ross syndrome is a degenerative disorder that progressively involves different fibre populations, starting with autonomic fibres and then involving the unmyelinated and myelinated sensory fibres. A careful clinical examination and simple bedside autonomic tests can confirm the diagnosis.