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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, View ORCID ProfileAdeline S.L. Ng
First published January 6, 2021, DOI: https://doi.org/10.1212/WNL.0000000000011496
Yongyao Kong
From the Departments of Neurology (Y.K., O.S.Q.K., Y.R.C., A.S.L.N.) and Neuroradiology (B.P.), National Neuroscience Institute, Singapore; Departments of Laboratory Medicine and Pathology and Neurology (A.M., A.Z.), Mayo Clinic College of Medicine, Rochester, MN; National Healthcare Group Eye Institute (C.F.C.), Tan Tock Seng Hospital; and Neuroscience and Behavioural Disorders (A.S.L.N.), Duke–NUS Medical School, Singapore.
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Andrew McKeon
From the Departments of Neurology (Y.K., O.S.Q.K., Y.R.C., A.S.L.N.) and Neuroradiology (B.P.), National Neuroscience Institute, Singapore; Departments of Laboratory Medicine and Pathology and Neurology (A.M., A.Z.), Mayo Clinic College of Medicine, Rochester, MN; National Healthcare Group Eye Institute (C.F.C.), Tan Tock Seng Hospital; and Neuroscience and Behavioural Disorders (A.S.L.N.), Duke–NUS Medical School, Singapore.
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Odelia S.Q. Koh
From the Departments of Neurology (Y.K., O.S.Q.K., Y.R.C., A.S.L.N.) and Neuroradiology (B.P.), National Neuroscience Institute, Singapore; Departments of Laboratory Medicine and Pathology and Neurology (A.M., A.Z.), Mayo Clinic College of Medicine, Rochester, MN; National Healthcare Group Eye Institute (C.F.C.), Tan Tock Seng Hospital; and Neuroscience and Behavioural Disorders (A.S.L.N.), Duke–NUS Medical School, Singapore.
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Yi Rong Chiew
From the Departments of Neurology (Y.K., O.S.Q.K., Y.R.C., A.S.L.N.) and Neuroradiology (B.P.), National Neuroscience Institute, Singapore; Departments of Laboratory Medicine and Pathology and Neurology (A.M., A.Z.), Mayo Clinic College of Medicine, Rochester, MN; National Healthcare Group Eye Institute (C.F.C.), Tan Tock Seng Hospital; and Neuroscience and Behavioural Disorders (A.S.L.N.), Duke–NUS Medical School, Singapore.
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Bela Purohit
From the Departments of Neurology (Y.K., O.S.Q.K., Y.R.C., A.S.L.N.) and Neuroradiology (B.P.), National Neuroscience Institute, Singapore; Departments of Laboratory Medicine and Pathology and Neurology (A.M., A.Z.), Mayo Clinic College of Medicine, Rochester, MN; National Healthcare Group Eye Institute (C.F.C.), Tan Tock Seng Hospital; and Neuroscience and Behavioural Disorders (A.S.L.N.), Duke–NUS Medical School, Singapore.
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Chee Fang Chin
From the Departments of Neurology (Y.K., O.S.Q.K., Y.R.C., A.S.L.N.) and Neuroradiology (B.P.), National Neuroscience Institute, Singapore; Departments of Laboratory Medicine and Pathology and Neurology (A.M., A.Z.), Mayo Clinic College of Medicine, Rochester, MN; National Healthcare Group Eye Institute (C.F.C.), Tan Tock Seng Hospital; and Neuroscience and Behavioural Disorders (A.S.L.N.), Duke–NUS Medical School, Singapore.
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Anastasia Zekeridou
From the Departments of Neurology (Y.K., O.S.Q.K., Y.R.C., A.S.L.N.) and Neuroradiology (B.P.), National Neuroscience Institute, Singapore; Departments of Laboratory Medicine and Pathology and Neurology (A.M., A.Z.), Mayo Clinic College of Medicine, Rochester, MN; National Healthcare Group Eye Institute (C.F.C.), Tan Tock Seng Hospital; and Neuroscience and Behavioural Disorders (A.S.L.N.), Duke–NUS Medical School, Singapore.
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Adeline S.L. Ng
From the Departments of Neurology (Y.K., O.S.Q.K., Y.R.C., A.S.L.N.) and Neuroradiology (B.P.), National Neuroscience Institute, Singapore; Departments of Laboratory Medicine and Pathology and Neurology (A.M., A.Z.), Mayo Clinic College of Medicine, Rochester, MN; National Healthcare Group Eye Institute (C.F.C.), Tan Tock Seng Hospital; and Neuroscience and Behavioural Disorders (A.S.L.N.), Duke–NUS Medical School, Singapore.
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  • ORCID record for Adeline S.L. Ng
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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
Neurology May 2021, 96 (19) e2454-e2455; DOI: 10.1212/WNL.0000000000011496

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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

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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.

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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.

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  • 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

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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