Several investigators have described the autonomic neuropathy in Sjögren's syndrome.1-3 However, only a few have documented the details of dysautonomia, or the pathology of nerves or of other organs such as eccrine sweat glands.3 We report on a patient with Sjögren's syndrome in whom dysautonomia was the dominant feature, and describe histological findings for the sural nerves and the eccrine glands.

A 39 year old woman visited our hospital because of frequent fainting attacks and diminished sweating. Her history indicated attacks of dizziness on standing since the age of 27. At the age of 30, she experienced a fainting attack on standing. At the age of 36, she noticed dry eyes and focal loss of sweating on the left forehead. She developed amenorrhea at the age of 27. The family history was unremarkable.

She was 164.5 cm tall and weighed 36 kg (body mass index 13.4). Neurological examination showed bilateral ptosis and isocoric pupils (3 mm in diameter; measured using infrared photography) with an irregular margin. The light reflex was absent and the accommodation reflex was tonic. Muscle tone and power were normal. The tendon reflexes were absent and plantar responses were flexor. Sensation and coordination were normal. The skin was generally dry, and spontaneous sweating was present …