Effective Short-Interval Intravenous Immunoglobulin Reinfusion in Steroid-Unresponsive Pediatric Myelin Oligodendrocyte Glycoprotein-Associated Disease.

Abstract

Management of the acute phase in steroid-unresponsive patients of myelin oligodendrocyte glycoprotein-associated disease (MOGAD) remains challenging, especially when there is no clinical improvement following a course of intravenous immunoglobulin (IVIG) which necessitates the need for reinfusion. The optimal timing of IVIG reinfusion in acute phase of MOGAD is a subject of debate. A 13-year-old South Indian boy presented with acute-onset paraparesis with urinary retention that progressed to quadriparesis within a week. Spine magnetic resonance imaging (MRI) spine revealed longitudinally extensive transverse myelitis from D2 to D7, whereas MRI brain showed subcortical white matter hyperintensities. Serum testing was strongly positive for myelin oligodendrocyte glycoprotein antibodies. Owing to persistent functional deficits following a course of IVIG, a reinfusion was administered at an interval of less than 2 weeks, shorter than the usual 3-4-week interval (corresponding to the half-life of IVIG) maintaining the presence and effectiveness of IVIG, leading to dramatic clinical improvement within a month.