Anti GFAP autoimmune encephalitis: management

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Anti GFAP autoimmune encephalitis: management. Diffuse periventricular white matter abnormalities. Fully referenced neurology checklist.

Magnetic resonance imaging (MRI): features

  • Diffuse periventricular white matter abnormalities
  • Linear perivascular enhancement: extending radially from the ventricles
  • Leptomeningeal enhancement
  • Longitudinally extensive myelopathy
  • Cerebellar atrophy
  • Hippocampal atrophy: case report
  • Reversible splenial lesion (RESLES)
  • Partially reversible FLAIR hyperintensities on the brainstem surface
  • Extensive corticospinal tract involvement
  • The trident sign
  • Orbital fat hyperintensity

Cerebrospinal fluid (CSF) features

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Other investigations

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Treatment

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References

  1. Fang B, McKeon A, Hinson SR, et al. Autoimmune glial fibrillary acidic protein astrocytopathy: a novel meningoencephalomyelitis. JAMA Neurol 2016; 73:1297-1307. 
  2. Flanagan EP, Hinson SR, Lennon VA, et al. Glial fibrillary acidic protein immunoglobulin G as biomarker of autoimmune astrocytopathy: analysis of 102 patients. Ann Neurol 2017; 81:298-309.
  3. Fang B, McKeon A, Hinson SR, Kryzer TJ, Pittock SJ, Aksamit AJ, Lennon VA. Autoimmune glial fibrillary acidic protein astrocytopathy: a novel meningoencephalomyelitis. JAMA Neurol 2016; 73:1297-1307.
  4. Zarkali A, Cousins O, Athauda D, Moses S, Moran N, Harikrishnan S. Glial fibrillary acidic protein antibody-positive meningoencephalomyelitis. Pract Neurol 2018; 18:315-319. 
  5. Iorio R, Damato V, Evoli A, et al. Clinical and immunological characteristics of the spectrum of GFAP autoimmunity: a case series of 22 patients. JNNP 2018; 89:138-146. 
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