Glial Fibrillary Acidic Protein (GFAP) Astrocytopathy Presenting as Mild Encephalopathy with Reversible Splenium

Charlotte Héraud1, Nicolas Capet2,3, Michaël Levraut3,4

  • 1Service de Neurologie, Centre Hospitalier Universitaire de Nice, 30 Voie Romaine, 06100, Nice, France. heraud.c@chu-nice.fr.

Neurology and Therapy
|November 29, 2021
PubMed
Abstract

Insights

This study presents a rare case of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy in an adult, uniquely associated with Mild Encephalitis with Reversible Splenium of corpus callosum lesions (MERS). This highlights GFAP astrocytopathy

Area of Science:

  • Neurology
  • Immunology
  • Neuroscience

Background:

  • Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a rare neurological disorder.
  • It typically manifests as meningoencephalomyelitis.

Purpose of the Study:

  • To report an atypical presentation of GFAP astrocytopathy.
  • To highlight the association with Mild Encephalitis with Reversible Splenium of corpus callosum lesions (MERS).

Main Methods:

  • Case report of a 26-year-old woman with progressive neurological symptoms.
  • Neuroimaging revealed MERS.
  • Autoimmune workup confirmed GFAP antibodies.

Main Results:

  • Diagnosis of GFAP astrocytopathy was established.
  • This represents the first reported adult case associating MERS and GFAP astrocytopathy.

Conclusions:

  • GFAP astrocytopathy can present atypically.
  • The association with MERS in adults is novel.
  • Diverse neurological symptoms of GFAP astrocytopathy may lead to misdiagnosis.