A review of chronic relapsing inflammatory optic neuropathy

Treatment and Relapse Prevention of Typical and Atypical Optic Neuritis

“…The main characteristic of patients with chronic relapsing inflammatory optic neuropathy (CRION) is the rapid and excellent response to corticosteroid therapy, as well steroid dependence, with relapses within weeks or months after the withdrawal of or a decrease in corticosteroids [ 185 ]. CRION requires careful consideration and differentiation from typical, demyelinating optic neuritis, since the treatment is entirely different, and the outcome without treatment is likely to be very poor.…”

“…For the time being, treatment recommendations are based on the activity of the disease and the clinical experience with related disorders, as no CRION-specific formal guidelines have been established yet. The general approach in the acute phase of the disease is the administration of IV methylprednisolone 1 mg/kg for 3–5 days, possibly with added IVIg or PLEX in severe cases, followed by oral steroids (1 mg/kg) with gradual tapering, as an abrupt withdrawal of treatment may lead to the irreversible worsening of visual acuity [ 185 , 188 ]. Given that relapses are common, the abrupt disruption of treatment should be avoided, and the minimal effective glucocorticoid dose be identified.…”

“…Azathioprine, rituximab, IVIg, cyclophosphamide, and methotrexate have been used for long-term and short-term treatment in single reports [ 136 ]. Natalizumab has also been employed [ 185 ]. Of course, if MOG or AQP4 seropositivity or another connective tissue disease can be identified, the therapeutic approach should be targeted at those disorders [ 188 ].…”