A 66-year-old man presented with a 1-year history of progressive cognitive decline, gait instability, and hearing loss. MRI of the brain showed extensive pachymeningeal thickening and enhancement with marked occlusion of intracranial dural venous sinuses (Figure 1). Lumbar puncture revealed pleocytosis at 140 cells/mm3 (96% lymphocytes) and elevated protein at 560 mg/dL. Serum syphilis screen, immunoglobulin G4, and proteinase 3 antibody were negative. Myeloperoxidase antibody was borderline positive. Systemic evaluation revealed evidence of aortitis and severe aortic regurgitation (Figure 2A). Dural biopsy was consistent with granulomatosis with polyangiitis (GPA).1 Special stains for microorganisms were negative (Figure 2, B–D). The patient was treated with intravenous methylprednisolone, followed by an oral prednisone taper. Rituximab was initiated.2 He returned in 2 months with near resolution of neurologic symptoms. Pachymeningitis can rarely be the initial manifestation of GPA, leading to cranial nerve impingement and venous outflow obstruction. Tissue biopsy and systemic evaluation are important in making the diagnosis.
(A, B) T1-contrasted weighted MRI showed diffuse pachymeningeal thickening and enhancement. (B) Enlarged diploic veins as accessory drainage pathways (arrow). Incidental fibrous dysplasia (arrowhead). (C) Diffuse stenosis of the venous sinuses on MR venogram (arrow). (D) Prominent ectasia of the optic nerve sheath on the left, suggesting intracranial hypertension.
(A) PET-CT showed increased fluorodeoxyglucose uptake at the root of the aorta (arrow). (B) Dural biopsy showed extensive necrosis, macrophages infiltration with vague granulomatous features, and microabscesses (asterisks). (C) Area marked with asterisk under 200× magnification. (D) Area marked with double asterisks under 400× magnification.
Author Contributions
X. Li: drafting/revision of the manuscript for content, including medical writing for content; major role in the acquisition of data; analysis or interpretation of data. D. Stitt: drafting/revision of the manuscript for content, including medical writing for content; major role in the acquisition of data; analysis or interpretation of data. G. Lanzino: drafting/revision of the manuscript for content, including medical writing for content; major role in the acquisition of data; analysis or interpretation of data. C. Giannini: drafting/revision of the manuscript for content, including medical writing for content; major role in the acquisition of data; analysis or interpretation of data. D. Dubey: drafting/revision of the manuscript for content, including medical writing for content; major role in the acquisition of data; study concept or design; analysis or interpretation of data. I.D. Carabenciov: drafting/revision of the manuscript for content, including medical writing for content; major role in the acquisition of data; study concept or design; analysis or interpretation of data.
Study Funding
No targeted funding reported.
Disclosure
X. Li and D. Stitt report no disclosures relevant to the manuscript. G. Lanzino has consulted for Nested Knowledge and Superior Medical Editors. C. Giannini reports no disclosures relevant to the manuscript. D. Dubey has consulted for UCB, Immunovant, Argenx, Arialys, and Astellas pharmaceuticals (All compensation for consulting activities is paid directly to Mayo Clinic); is a named inventor on filed patent that relates to KLHL11 as a marker of autoimmunity and germ cell tumor; has patents pending for LUZP4-IgG and cavin-4-IgG as markers of neurologic autoimmunity; and has received funding from the DOD (CA210208 & PR220430). I.D. Carabenciov reports no disclosures relevant to the manuscript. Go to Neurology.org/N for full disclosures.
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 & Fellow Section Editor Whitley Aamodt, MD, MPH.
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- Received March 3, 2023.
- Accepted in final form June 26, 2023.
- © 2023 American Academy of Neurology
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