Adie’s tonic pupil in sjogren's syndrome

Abstract

Adie’s tonic pupil is a neurological disorder more common in women. It is characterized by persistent unilateral mydriasis. Tonic pupil constricts poorly to light but reacts better to accommodation. It is caused by parasympathetic ciliary ganglion damage and is characterized by hypersensitivity to muscarinic receptor agonists (pilocarpine). Most cases are idiopathic, though it can be caused by autoimmune diseases, tumors, trauma, and surgery. We report a case of a 39-year-old woman with a past medical history for migrainous headache, left knee and bilateral femoral head osteonecrosis, anemia and arthritis. She was diagnosed with sjogren syndrome based on symptoms of mouth and eyes dryness, positive serum anti-RO/SSA and ANA antibodies and labial salivary gland biopsy showing lymphocytic sialadenitis. She presented with progressive vision loss in the right eye. The initial examination showed anisocoria, an afferent pupillary defect and a sector pupil palsy of the right eye. Ophthalmoscopic evaluation, visual acuity and field testing were normal. Visual-evoked potentials (VEPs) were normal. Ankle jerk reflexes were absent. The diagnosis of Adie’s tonic pupil was confirmed with rapid miotic response of the affected pupil to 0.125% pilocarpine drop. The patient was treated with Pilocarpine drops and had slightly improved symptoms. Adie’s tonic pupil is a benign, mostly idiopathic syndrome caused by parasympathetic denervation. However, it can be associated with infectious, inflammatory, malignant, or autoimmune pathologies. Systemic conditions associated with tonic pupils are rare. This is, as far as we know, the first case reported of a sjogren syndrome associated with tonic pupil.