A 78-year-old healthy woman presented with abulia, aphasia, and echopraxia for 1 week. MRI of the brain with contrast (Figure 1) revealed radial perivascular enhancement perpendicular to the ventricles. CSF showed 16 white blood cells (normal 0–5/μL), 1.18 g/L proteins (normal <0.45 g/L), CD4/CD8 ratio of 14.7, and no neoplastic cells. Extensive autoimmune, infectious, and neoplastic workup was unremarkable. CSF and serum glial fibrillary acidic protein (GFAP) immunoglobulin G were negative. Brain biopsy (Figure 2) confirmed neurosarcoidosis. After corticosteroid treatment, MRI was normalized (Figure 1) and the patient substantially improved; however, she remained disabled with a multidomain cognitive impairment.
Figure 1. Postgadolinium Axial and Sagittal T1-Weighted Images at Baseline Reveal Linear Perivascular Enhancement (A, B), While Postgadolinium Axial and Sagittal T1-Weighted Images at 3 Months After Corticosteroid Treatment Show Significant Improvement of the Previous MRI Pattern (C, D).
Figure 2. Brain Biopsy in the Right Frontal Lobe Reveal White Matter Inflammatory Granulomatous Lesion With a Histiocytic Center and a Ring of T Lymphocytes (Predominantly CD4+), Associated With Small Inflammatory Macrophagic Lesions Centered to White Matter Vessels.
No fibrinoid necrosis or microorganisms (A and B: hematoxylin and eosin; C: CD68; D: CD3).
This linear radial periventricular enhancement pattern on MRI brain is a hallmark of autoimmune GFAP astrocytopathy1 and can also be seen with intravascular lymphoma, CNS vasculitis, and neurosarcoidosis,2 which are advisable to include in the differential diagnosis of patients with this MRI pattern, negative GFAP workup, and no evidence of malignancy.
Footnotes
Teaching slides links.lww.com/WNL/D68
Author Contributions
A. Costa: drafting/revision of the manuscript for content, including medical writing for content; major role in the acquisition of data; study concept or design. C. Silva: drafting/revision of the manuscript for content, including medical writing for content; major role in the acquisition of data. R. Taipa: major role in the acquisition of data; analysis or interpretation of data. J.P. Gabriel: drafting/revision of the manuscript for content, including medical writing for content. M. Mendes: drafting/revision of the manuscript for content, including medical writing for content; major role in the acquisition of data.
Study Funding
No targeted funding reported.
Disclosure
The authors report no relevant disclosures. Go to Neurology.org/N for full disclosures.
References
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