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September 07, 2021; 97 (10) Resident & Fellow Section

Teaching NeuroImage: Papilledema and Pachymeningitis

An Atypical Presentation of Granulomatosis With Polyangiitis

Christopher Radcliffe, Kunal Potnis, Teng J. Peng, Eliza DeCroce-Movson, Kathryn Zuchowski, Adeniyi Fisayo
First published May 12, 2021, DOI: https://doi.org/10.1212/WNL.0000000000012156
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A 53-year-old man presented with worsening headaches and progressive vision loss. His medical history was notable for biopsy-proven granulomatosis with polyangiitis (GPA), which was diagnosed after an episode of fever and hemoptysis years prior to the current presentation. Ophthalmologic examination was remarkable for bilateral papilledema (figure 1). Brain MRI with and without contrast demonstrated pachymeningitis and ventriculomegaly. Following ventriculoperitoneal shunt placement, there was resolution of headaches, papilledema, and ventriculomegaly (figure 2). The patient was subsequently treated with rituximab. Pachymeningitis—pathologic thickening of the dura mater—stands as a rare manifestation of GPA.1 The most common manifestation of pachymeningitis is cranial nerve paralysis, but it can also hinder venous sinus drainage and cause a communicating hydrocephalus.2

Figure 1
Figure 1 Pachymeningitis, Ventriculomegaly, and Papilledema in a Patient With Granulomatosis With Polyangiitis

(A) Postcontrast T1 sequence MRI axial view demonstrates meningeal enhancement and ventriculomegaly. (B) Postcontrast T1 sequence MRI coronal view. (C) Elevated optic disc with blurred margins and surrounding flame hemorrhages consistent with papilledema.

Figure 2
Figure 2 Resolution of Ventriculomegaly and Papilledema After Ventriculoperitoneal Shunt Placement

(A) Postcontrast T1 sequence MRI axial view demonstrates persistent meningeal enhancement and normal-sized ventricles. (B) Postcontrast T1 sequence MRI coronal view. (C) Resolution of papilledema.

Study Funding

No targeted funding reported.

Disclosure

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

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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/B409

References

  1. 1.↵
    1. Smolenska Z,
    2. Masiak A,
    3. Zdrojewski Z
    . Hypertrophic pachymeningitis as an important neurological complication of granulomatosis with polyangiitis. Reumatologia. 2018;56(6):399-405.
    OpenUrl
  2. 2.↵
    1. Scarrow AM,
    2. Segal R,
    3. Medsger TA Jr.,
    4. Wasko MC
    . Communicating hydrocephalus secondary to diffuse meningeal spread of Wegener's granulomatosis: case report and literature review. Neurosurgery. 1998;43(6):1470-1473.
    OpenUrlCrossRefPubMed

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