A case of Leber's neuroretinitis

Background: Described in 1916 by Theodore Leber, this rare syndrome charact erized by low visual acuity, papillary edema, and a macular star (dry exsud ates) occurs classically in the 30 to 40 year age range but also as frequen tly in children. The visual prognosis is excellent. An infectious cause is found in almost all cases. Case report: A 34-year-old man consulted for declining visual acuity of the right eye which began suddenly without pain and was preceded by a flu-like syndrome lasting two weeks. Visual acuity on the right side was 2/10 P6, t here was optic disk edema associated with dry macular exsudate (macular sta r) and a paleness of the posterior pole progressing to the periphery and re adily visualized on the angiography, and a central scotome, but no dyschrom atopsy. The left eye was strictly normal. Laboratory tests showed an erythr ocyte sedimentation rate at 45, C reactive protein at 61, normal red cell c ount and minimal transaminase elevation. Spinal tap showed: 3 elements/mm(3 ), protein 0.28g/l, 18% gammaglobulins. Serology tests were negative. Brain imaging was normal. Results: Corticosteroid flashes for three days were initiated and the patie nt was seen again two weeks later with clear clinical improvement. Visual a cuity was 6/10 P2 with considerable resorption of the edema. Discussion: Th is case is a typical illustration of acute Leber's neuroretinititis, probab ly caused by viral infection. The cerebrospinal fluid tests and brain imagi ng ruled out multiple sclerosis, and serology tests for syphilis, tuberculo sis, Lyme disease and possible parasite infection were negative. Conclusion: Acute lever's neuroretinitis is an uncommon condition which mus t be distinguished from inflammatory optic neuropathy, particularly in mult iple sclerosis which has a very different prognosis and clinical course.