Bilateral sequential optic neuropathy as the initial manifestation of Sjögren syndrome

Neuro-ophthalmic findings are uncommon in the setting of Sjögren syndrome. We report the case of a patient with bilateral, sequential optic neuropathy as the initial manifestation of Sjögren syndrome.

A 38-year-old male presented with sudden painless visual loss in his left eye in May 2005. Fundus examination revealed a left swollen optic disk. Magnetic resonance imaging (MRI) revealed a left optic nerve lesion. Elevated titres of autoantibodies (ANA, anti-SSA, anti-SSB) were found, suggestive of Sjögren syndrome. In January 2006, he presented with painful sudden visual loss in the right eye. Fundus examination revealed a right swollen optic disk and left optic nerve atrophy. MRI was normal. Other aetiologies were ruled out.

Each episode was treated with intravenous methylprednisolone (1 g/day during 3 days), followed by oral prednisone (1 mg/kg/day). Moderate improvement of vision ensued in both eyes.

Atypical presentation of an optic neuropathy must raise the suspicion of an unusual aetiology. Our case illustrates how a bilateral sequential optic neuropathy in an otherwise healthy patient can result from an unusual inflammatory aetiology: primary Sjögren syndrome.