Devic's neuromyelitis Optica treated with intravenous gamma globulin (IVIG)

Background: Devic's syndrome is a demyelinating disease of the spinal cord and optic nerves. It tends to have a poor prognosis, probably due to the occurrence of necrosis within lesions. There is no proven effective treatment although relapses are commonly treated with corticosteroids and people with recurrent attacks may be managed with chronic immune suppressing treatments. Intravenous gamma globulin (IVIG) and plasma exchange are reasonable treatment options because Devic's syndrome is believed to be antibody mediated. We report two patients of Devic's syndrome that stabilized following initiation of monthly IVIG. Patient 1: A 42-year-old woman with a 23 year history of Devic's syndrome continued to have frequent attacks of optic neuritis unresponsive to daily corticosteroids and azathioprine. Since initiation of monthly IVIG 5 1/2 years ago she has had no further definite attacks. She has also noted minimal improvement in color perception. Patient 2: A 58-year-old woman with a three year history of Devic's syndrome experienced five attacks during the first 16 months of disease. Monthly IVIG was associated with complete cessation of relapses and significantly improved neurological status over one year of treatment. Conclusions: Because active Devic's disease often results in severe, permanent neurological impairment, preventive intervention should be considered. These cases suggest that IVIG may be effective in preventing attacks and possibly in enhancing neurological recovery. Randomized controlled trials will be needed to confirm this and to determine optimal dosing and treatment duration.