A 21-year-old man with headache, vomiting, and limb weakness presented to the clinic 2 years ago. An examination showed paresthesia and weakness in left upper and lower limbs. An examination of the brain MRI demonstrated a large space-occupying lesion with ring enhancement and compression of the right fronto-tempo-parietal lobes (Figure 1, A and B). The patient underwent surgery for a presumed glioblastoma. Pathologic examination revealed primary central vasculitis (PCNSV) without neoplasm (Figure 2). His screening workup for systemic vasculitis showed negative results. Symptoms improved after a corticosteroid taper. After stopping immunosuppressive therapy for 1 year, new lesions were found again in the right frontotemporal lobe (Figure 1, C and D). Corticosteroids and mycophenolate mofetil were given, and the patient's symptoms significantly improved and lesions on MRI had subsided significantly (Figure 1, E and F). MRI findings of PCNSV frequently present as nonspecific white matter lesions.1 It can mimic glioblastoma,2 CNS lymphoma, and tumefactive multiple sclerosis.1

Figure 1 MRI of the Brain

Brain MRI demonstrates hyperintense irregular mass within the right frontotemporal and parietal lobes with perifocal edema and ring enhancement (A–B). A lesion appeared in the right frontotemporal lobe and corpus callosum, and the anterior horn of the right ventricle was significantly compressed (C–D). MRI lesion largely disappeared after immunosuppressant therapy (E–F).

Figure 2 Pathology Slide

H&E (A) showed necrosis of small blood vessels with perivascular infiltrates of lymphocytes. Anti-CD68 immunostain (B) demonstrating macrophage expression. H&E = hematoxylin & eosin.

Study Funding

The authors report no targeted funding.

Disclosure

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

Author Contributions

H. Sun: drafting/revision of the article for content, including medical writing for content; major role in the acquisition of data; study concept or design; and analysis or interpretation of data. S. Zhang: analysis or interpretation of data. T. Yu: analysis or interpretation of data. D. Zhou: drafting/revision of the article for content, including medical writing for content; major role in the acquisition of data. J. Li: drafting/revision of the article for content, including medical writing for content; major role in the acquisition of data; and analysis or interpretation of data.

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.

Submitted and externally peer reviewed. The handling editor was Resident and Fellow Section Editor Whitley Aamodt, MD, MPH.

Teaching slides links.lww.com/WNL/C640

Received July 22, 2022.Accepted in final form December 20, 2022.Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.