Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy Presenting with Slowly Progressive Myelitis and Longitudinally Extensive Spinal Cord Lesions

Intern Med. 2020 Nov 1;59(21):2777-2781. doi: 10.2169/internalmedicine.5074-20. Epub 2020 Jul 14.

Abstract

We report a 65-year-old man with autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) who presented with gait disturbance that he had experienced for approximately half a year. On neurological examination, he displayed spastic paraplegia and autonomic dysfunctions including dysuria and constipation. Spinal cord magnetic resonance imaging showed longitudinally extensive spinal cord lesions (LESCLs) extending from the cervical to the thoracic cords. The patient was negative for anti-myelin oligodendrocyte glycoprotein and anti-aquaporin 4 antibodies. Treatment with corticosteroids and intravenous immunoglobulin resulted in a clinical improvement. It is important to distinguish GFAP-A from slowly progressive myelitis with LESCLs.

Keywords: astrocytopathy; autoantibody; glial fibrillary acidic protein (GFAP); longitudinally extensive spinal cord lesion (LESCL); myelitis; neuromyelitis optica spectrum disorders (NMOSDs).

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Astrocytes / pathology*
  • Autoantibodies / adverse effects*
  • Demyelinating Autoimmune Diseases, CNS / diagnosis*
  • Demyelinating Autoimmune Diseases, CNS / immunology
  • Demyelinating Autoimmune Diseases, CNS / physiopathology*
  • Glial Fibrillary Acidic Protein / adverse effects*
  • Glial Fibrillary Acidic Protein / immunology*
  • Humans
  • Male
  • Neuromyelitis Optica / diagnosis
  • Neuromyelitis Optica / immunology
  • Neuromyelitis Optica / physiopathology*

Substances

  • Autoantibodies
  • Glial Fibrillary Acidic Protein