Background: Described by Theodore Leber in 1916, this syndrome is characterized by unilateral visual loss, optic disc swelling and a stellate pattern of exudative deposits in the macula.
Material and methods: Two young adults were examined for sudden unilateral visual loss preceded by a flu-like syndrome. Fundus examination revealed a swollen optic disc and a macular star, visualized on fluorescein angiography. Visual field, laboratory tests and cranial and orbital tomodensitometry were performed.
Results: Visual field examination showed a centrocecal scotoma. Laboratory tests showed inflammatory syndrome in one case. Serology testing (leptospirosis, cat scratch disease, toxoplasmosis and syphilis, etc.) were negative. Tomodensitometry of the brain and orbits gave normal results. One of the patients was treated with intravenous steroid boluses; the other received no treatment. A few weeks after the beginning of symptoms, the swelling of the optic disc seemed to have declined, leaving the macular star unchanged.
Conclusion: These two cases typically describe Leber's idiopathic stellate neuroretinitis. The prognosis for visual recovery is usually reported to be excellent, but visual sequelae have been described.