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November 26, 2019; 93 (22) Resident & Fellow Section

Teaching NeuroImages: Autoimmune glial fibrillary acidic protein meningoencephalomyelitis

Paula K.J. Lee, Juan E. Small, Michal Vytopil
First published November 25, 2019, DOI: https://doi.org/10.1212/WNL.0000000000008562
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A 73-year-old woman with myelodysplastic syndrome presented with 2 months of decline in cognition and mobility, accompanied by headache and weight loss. Examination revealed a somnolent woman with symmetric cogwheel rigidity, bradykinesia, arm myoclonus, and hyperreflexia. Brain MRI showed leptomeningeal, linear/stippled parenchymal, and ependymal enhancement (figure 1). Spine MRI demonstrated nonenhancing cervicothoracic cord signal change and circumferential enhancement of conus medullaris with central canal enhancement (figure 2). CSF was inflammatory with glial fibrillary acidic protein (GFAP)–immunoglobulin G (IgG). The patient responded promptly to IV methylprednisolone. GFAP-IgG identifies a severe but highly corticosteroid-responsive autoimmune meningoencephalomyelitis with distinctive MRI findings.1,2

Figure 1
Figure 1 Axial T1 postcontrast brain MRI

Leptomeningeal, linear/stippled parenchymal, and ependymal postgadolinium enhancement (arrows).

Figure 2
Figure 2 Thoracic sagittal T1 postcontrast MRI

Leptomeningeal (white arrows) and central canal enhancement (black arrows) throughout the cord (A) and conus medullaris (B). Axial T1 postcontrast image (C) with peripheral leptomeningeal enhancement and central dot of central spinal canal enhancement.

Study funding

No targeted funding reported.

Disclosure

The authors report no disclosures relevant to the manuscript. Go to Neurology.org/N for full disclosures.

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Footnotes

  • Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

  • Teaching slides links.lww.com/WNL/B2

References

  1. 1.↵
    1. Fang B,
    2. McKeon A,
    3. Hinson SR, et al
    . Autoimmune glial fibrillary acidic protein astrocytopathy: a novel meningoencephalomyelitis. JAMA Neurol 2016;73:1297–1307.
    OpenUrl
  2. 2.↵
    1. Flanagan EP,
    2. Hinson SR,
    3. 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.
    OpenUrl

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