gms | German Medical Science

Chronic inflammatory optic neuropathies (CION) are characterized by uni or bilateral visual loss, progressive or relapsing, leading to permanent visual impairment. CION is observed in multiple sclerosis, in systemic inflammatory diseases (sarcoïdosis, lupus erythematosus, Devic's disease). When CION is not associated with a systemic or a neurological disease, it is often associated with biological signs of dysimmunity such as antinuclear antibodies (FAN) and has been called auto-immune optic neuropathy. CION has no recognized treatment and despite steroids therapy there is a continuing decrease in visual acuity and cytotoxic drugs are often necessary to maintain vision.

We report clinical findings and treatment of four patients suffering from chronic inflammatory optic neuropathy and compare the results to the few series reported in the litterature.

There were three women and one man, mean age 31,2 years old, with bilateral visual loss (8 eyes), and more than one year follow-up. Cerebral MRI was normal in all cases. Medullar MRI, lumbar puncture and laboratory evaluation (FAN, ACE, anticardiolipin antibodies.) were performed in all patients. In all cases steroids were unable to maintain good vision. One case was treated by mitoxantrone and three with cyclophosphamide. Three patients improved with immunosuppresive treatment and one was stable. Only two eyes of two different patients had final acuity less than 0,05.

These patients with CION must be early recognized, followed carefully to facilitate the appropriate therapy. Further studies, including more patients will be necessary to evaluate immunosuppresive drugs.