Walsh & Hoyt: Treatment

There is no proven effective treatment for neuromyelitis optica. Supportive care is crucial to ensure survival in patients with severe myelitis. The use of high-dose IV corticosteroids may lessen the severity of the attack and increase the speed of recovery of both visual and motor function. Some patients, however, may become steroid-dependent and experience relapses of symptoms with tapering of dosage. Frohman et al. reported the case of a 15-year-old boy who developed typical neuromyelitis optica when he was 12 years old. His vision recovered, but he experienced recurrent attacks of bilateral, simultaneous optic neuritis over the next 3 years and became steroid-dependent. Administration of IV-Ig allowed discontinuation of steroids without further ophthalmologic or neurologic deterioration or recurrent disease. Plasma exchange was also associated with improvement in a small case series (10 patients). Interferons and immunosuppressants, including azathioprine, have been tried, but prospective data from controlled studies demonstrating efficacy are lacking. Given the occurrence of spontaneous improvement in neuromyelitis optica, randomized controlled trials of therapies are needed to establish treatment recommendations.