Acute Disseminated Encephalomyelitis (ADEM) and Increased Intracranial Pressure Associated With Anti-Myelin Oligodendrocyte Glycoprotein Antibodies

Pediatr Neurol. 2019 Oct:99:64-68. doi: 10.1016/j.pediatrneurol.2019.03.009. Epub 2019 Mar 22.

Abstract

Background: Antibodies to the myelin oligodendrocyte glycoprotein (MOG) have been identified in about 40% of children with acute disseminated encephalomyelitis (ADEM). The objective of this report is to describe three individuals with fulminant ADEM complicated by increased intracranial pressure associated with the presence of the anti-MOG antibodies.

Methods: This is a retrospective case series. Informed consent was obtained from the concerned patients or caregivers.

Results: High intracranial pressure associated with ADEM in the presence of MOG antibodies can result in cerebral edema, herniation, prolonged hospital stay (average intensive care unit stay: 22 days, average hospital stay: 50.6 days), and long-term disability.

Conclusion: Increased intracranial pressure complicating MOG antibody-related ADEM is a unique finding in our cases. This can complicate the clinical picture of ADEM and confers high morbidity. Long-term immunosuppression is warranted in selected cases with persistent seropositivity.

Keywords: Acute disseminated encephalomyelitis; Cerebral edema; Herniation; Intracranial pressure; Myelin oligodendrocyte Glycoprotein; Optic neuritis.

Publication types

  • Case Reports

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Anti-Inflammatory Agents / therapeutic use
  • Autoantibodies / blood
  • Autoantibodies / immunology*
  • Autoantigens / immunology*
  • Brain Damage, Chronic / etiology
  • Brain Edema / etiology
  • Cardiovascular Diseases / etiology
  • Child
  • Child, Preschool
  • Combined Modality Therapy
  • Cyclophosphamide / therapeutic use
  • Drug Therapy, Combination
  • Encephalomyelitis, Acute Disseminated / blood
  • Encephalomyelitis, Acute Disseminated / complications
  • Encephalomyelitis, Acute Disseminated / immunology*
  • Encephalomyelitis, Acute Disseminated / therapy
  • Epilepsy / etiology
  • Female
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Infections / complications
  • Intracranial Hypertension / blood
  • Intracranial Hypertension / drug therapy
  • Intracranial Hypertension / immunology*
  • Male
  • Myelin-Oligodendrocyte Glycoprotein / immunology*
  • Optic Neuritis / etiology
  • Paresis / etiology
  • Plasmapheresis
  • Retrospective Studies
  • Rituximab / therapeutic use

Substances

  • Adrenal Cortex Hormones
  • Anti-Inflammatory Agents
  • Autoantibodies
  • Autoantigens
  • Immunosuppressive Agents
  • Myelin-Oligodendrocyte Glycoprotein
  • Rituximab
  • Cyclophosphamide