Share

May 11, 2021; 96 (19) Resident & Fellow Section

Teaching NeuroImages: Linear Radial Periventricular Enhancement in Glial Fibrillary Acidic Protein Astrocytopathy

Yongyao Kong, Andrew McKeon, Odelia S.Q. Koh, Yi Rong Chiew, Bela Purohit, Chee Fang Chin, Anastasia Zekeridou, Adeline S.L. Ng
First published January 6, 2021, DOI: https://doi.org/10.1212/WNL.0000000000011496
Full PDF
Citation
Permissions

Make Comment

See Comments

Downloads
299

Share

A 48-year-old woman presented with asymptomatic painless optic disc swelling, seizures, cognitive decline, and weakness over 3 months. She had an upper motor neuron pattern of grade 4/5 weakness in all 4 limbs. Brain MRI revealed striking linear radial perivascular enhancement in the deep white matter1,2 (figure, A and B); MRI spine revealed longitudinally extensive C3-to-conus cord T2 hyperintensity and T1 enhancement2 (figure, C and D). CSF demonstrated glial fibrillary acidic protein (GFAP) pattern by indirect immunofluorescence with GFAPα specificity confirmed by cell-based assay. The patient improved clinically (figure, E and F) with steroid treatment. Linear/radial perivascular enhancement in the deep white matter prompts consideration of a corticosteroid-responsive meningoencephalomyelitis termed autoimmune GFAP astrocytopathy.1

Figure
Figure Baseline and Interval MRI Features in Glial Fibrillary Acidic Protein Astrocytopathy After Immunosuppressive Treatment

(A) Baseline T2-weighted deep white matter hyperintensities. (B) Baseline contrast-enhanced T1-weighted linear/radial perivascular enhancement. (C) Baseline T2-weighted dorsal cord focal hyperintensity. (D) Contrast-enhanced T1-weighted leptomeningeal enhancement over conus. (E) Interval T2-weighted progression/confluence of hyperintensities in periventricular and deep white matter with background involution. (F) Corresponding resolution of radial perivascular enhancement.

Study Funding

No targeted funding reported.

Disclosure

A. McKeon serves as an editorial board member of Neurology®: Neuroimmunology and Neuroinflammation; received research support from Medimmune, Euroimmun, Grifols, and Alexion; and has patents pending for Septin 5, GFAP, PDE10A, Kelch-11, and MAP1B as markers of neurologic autoimmunity and paraneoplastic disorders. A. Zekeridou has a patent pending for PDE10A-IgG paraneoplastic autoimmunity. Y. Kong, O.S.Q. Koh, Y.R. Chiew, B. Purohit, C.F. Chin, and A.S.L. Ng report no disclosures. Go to Neurology.org/N for full disclosures.

Appendix Authors

Table1

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/B306

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

Disputes & Debates: Rapid online correspondence

No comments have been published for this article.
Comment

NOTE: All authors' disclosures must be entered and current in our database before comments can be posted. Enter and update disclosures at http://submit.neurology.org. Exception: replies to comments concerning an article you originally authored do not require updated disclosures.

  • Stay timely. Submit only on articles published within 6 months of issue date.
  • Do not be redundant. Read any comments already posted on the article prior to submission.
  • 200 words maximum.
  • 5 references maximum. Reference 1 must be the article on which you are commenting.
  • 5 authors maximum. Exception: replies can include all original authors of the article.
  • Submitted comments are subject to editing and editor review prior to posting.

More guidelines and information on Disputes & Debates

Compose Comment

More information about text formats

Plain text

  • No HTML tags allowed.
  • Web page addresses and e-mail addresses turn into links automatically.
  • Lines and paragraphs break automatically.
Author Information
NOTE: The first author must also be the corresponding author of the comment.
First or given name, e.g. 'Peter'.
Your last, or family, name, e.g. 'MacMoody'.
Your email address, e.g. higgs-boson@gmail.com
Your role and/or occupation, e.g. 'Orthopedic Surgeon'.
Your organization or institution (if applicable), e.g. 'Royal Free Hospital'.
Publishing Agreement
NOTE: All authors, besides the first/corresponding author, must complete a separate Disputes & Debates Submission Form and provide via email to the editorial office before comments can be posted.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.

Vertical Tabs

You May Also be Interested in

Back to top
Advertisement

Related Articles

Topics Discussed

Alert Me