Plasmapheresis as at Treatment Option in Anti-MOG–Related Neurological Disorders with Fulminant Progress

Introduction: Plasmapheresis is a blood separation technology, in which harmful antibodies can be removed from patients by an extracorporeal procedure. In CNS disorders caused by anti-MOG-antibody, steroids or IVIG have shown to be effective. In selected cases, steroids and IVIG show no immediate effect. Therefore, in case with a fulminant progress, plasmapheresis is a promising option.

Case Report: Case 1: A 13-year-old girl suffered from unilateral relapsing optic neuritis. Before anti-MOG-antibodies were detected, she was treated with steroids. Due to side effects, steroids were replaced by glatiramer acetate. While tapering steroids, she rapidly developed a complete visual loss. Again, she was treated with high steroids with no improvement. Plasmapheresis was initiated leading to significant improvement after the third cycle. The patient regained a normal visual acuity (1,0) and receives now subcutaneous immunoglobulin weekly. Case 2: A 3.8-year-old boy with symptoms of ADEM, associated with increased anti-MOG-antibody: 1:640. Restitutio ad integrum under steroids. Brain MRI 5 months later showed small lesion in the white matter (right hemisphere) and anti-MOG-antibody decreasing to 1:160 were revealed. At the age of 7.5 years, the patient had a second foudroyant episode of a complete transverse myelitis. Large lesions were detectable in brain and spinal MRI. Anti-MOG-antibody 1:2560. Steroids and IVIG showed no effect. He developed complete tetraplegia and signs of encephalopathy. Plasmapheresis was initiated, leading to recovery after the fourth cycle.

Conclusion: Similar to therapeutic strategies in severe GBS or antibody-positive Myasthenia gravis, plasmapheresis is a promising treatment of option in rapidly progressive forms of anti-MOG-antibody–positive disease of the CNS.