B-cell depletion is ineffective in a patient with granulomatous optic neuropathy

A 49-year-old woman had sudden visual loss in her left eye associated with retrobulbar pain and decrease in visual evoked potential amplitude. With suspected optic neuritis, the patient was treated successfully with a 5-day therapy of methylprednisolone (MP) infusion of 1 g per day (figure 1A). Within several days after MP, partial visual loss reoccurred. MRI showed increased signal and contrast enhancement of the left optic nerve (figure 1, B and C); further diagnostic workup including cerebral and spinal MRI and CSF analysis with oligoclonal bands and soluble Il-2 receptor was normal. Serum antibody screening for aquaporin 4, myelin oligodendrocyte glycoprotein (MOG) and myelin-associated glycoprotein (MAG) was negative. Chest CT did not show signs of hilar adenopathy, interferon-gamma release assays for tuberculosis were negative, and ophthalmologic examination excluded optic retinitis. Optic neuritis relapsed 3 times within the next 4 months and was treated with IV MP and with the suspected diagnosis of atypical relapsing optic neuritis (RON) therapy with azathioprine and overlapping oral MP was initiated (figure 1A). After a stable period of 8 months, azathioprine had to be discontinued because of severe leucopenia. Thereafter, another series of relapses with complete visual loss of the left eye occurred that was refractory to a total dose of 15 g MP and additional cycles of plasma exchange after initial partial relief. Since another approach with azathioprine ...
Source: Neurology Neuroimmunology and Neuroinflammation - Category: Neurology Authors: Tags: Optic nerve, Visual loss, Optic neuritis; see Neuro-ophthalmology/Optic Nerve Clinical/Scientific Notes Source Type: research