Overlapping Epstein-Barr virus encephalitis and autoimmune glial fibrillary acidic protein astrocytopathy

J Neuroimmunol. 2023 Sep 15:382:578174. doi: 10.1016/j.jneuroim.2023.578174. Epub 2023 Aug 7.

Abstract

We describe three cases of overlapping Epstein-Barr virus (EBV) Encephalitis and Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy (GFAP-A). The three cases all presented with initial symptoms of fever, headache, coma, and posture tremor of the upper limbs, then followed by limb weakness and dysuria. All of the three cases were on ventilators. Case 1 and 2 improved dramatically after intravenous methylprednisoloneand immunoglobulin treatment. However, case 3 presented dyspneic, and died from gastrointestinal hemorrhage. The GFAP-A triggered by EBV intracranial infection could initially masquerade as EBV encephalitis only, and the detection of GFAP antibody is essential for differentiation.

Keywords: Acute disseminated encephalomyelitis; Autoimmune glial fibrillary acidic protein astrocytopathy; Epstein-Barr virus encephalitis.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Antibodies
  • Astrocytes* / immunology
  • Astrocytes* / metabolism
  • Autoantibodies
  • Autoimmune Diseases of the Nervous System* / complications
  • Autoimmune Diseases of the Nervous System* / diagnosis
  • Autoimmune Diseases of the Nervous System* / therapy
  • Diagnosis, Differential
  • Encephalitis* / complications
  • Encephalitis* / immunology
  • Encephalitis* / therapy
  • Epstein-Barr Virus Infections* / complications
  • Epstein-Barr Virus Infections* / therapy
  • Glial Fibrillary Acidic Protein* / genetics
  • Glial Fibrillary Acidic Protein* / immunology
  • Glucocorticoids / therapeutic use
  • Herpesvirus 4, Human
  • Humans
  • Immunoglobulins, Intravenous
  • Methylprednisolone / therapeutic use

Substances

  • Antibodies
  • Autoantibodies
  • Glial Fibrillary Acidic Protein
  • Immunoglobulins, Intravenous
  • Methylprednisolone
  • Glucocorticoids