The role of plasmapheresis in severe acute disseminated encephalomyelitis with clinical findings of transverse myelitis

Abstract

Introduction: Acute disseminated encephalomyelitis is a rare acute demyelinating disease of the central nervous system (CNS). The pathogenesis remains unclear but is suspected to be autoimmune. High doses of methylprednisolone (HDMP) are currently considered standard of treatment. Plasmapheresis (PE) is typically given in steroid refractory cases. There is currently limited evidence supporting its use in ADEM.

Materials and methods: We report a 16-year-old girl with ADEM who improved rapidly after initiating PE.

Results: The patient presented with acute onset of multifocal CNS symptoms, including encephalopathy, requiring intensive care unit management. Despite HDMP administration, her clinical condition continued to deteriorate. PE was therefore initiated on the same day as HDMP. Her clinical condition improved significantly following the first session. She was extubated and discharged from the intensive care unit the following day.

Conclusion: HDMP combined with PE may be an effective first-line treatment in patients with fulminant ADEM.

Keywords: ADEM; acute disseminated encephalomyelitis; children; plasmapheresis; therapeutic plasma exchange.