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
(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
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
- © 2021 American Academy of Neurology
Disputes & Debates: Rapid online correspondence
REQUIREMENTS
If you are uploading a letter concerning an article:
You must have updated your disclosures within six months: http://submit.neurology.org
Your co-authors must send a completed Publishing Agreement Form to Neurology Staff (not necessary for the lead/corresponding author as the form below will suffice) before you upload your comment.
If you are responding to a comment that was written about an article you originally authored:
You (and co-authors) do not need to fill out forms or check disclosures as author forms are still valid
and apply to letter.
Submission specifications:
- Submissions must be < 200 words with < 5 references. Reference 1 must be the article on which you are commenting.
- Submissions should not have more than 5 authors. (Exception: original author replies can include all original authors of the article)
- 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.
- Submitted comments are subject to editing and editor review prior to posting.