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Pial involvement in Wegener's granulomatosis shown on MRI
A O Nusbaum1, S Morgello, S W Atlas
1Department of Radiology, Mount Sinai School of Medicine, New York, NY 10029, USA.
Abstract:
Involvement of the brain and meninges is rare in Wegener's granulomatosis (WG); it has been reported in 1.2-8 % of patients. Meningeal involvement in WG has been reported in imaging as being confined to the dura mater, and is thought to represent granulomatous infiltration. We present a case of WG with abnormal pial enhancement and involvement of the perivascular spaces on MRI, pathologically proven to represent granulomatous infiltration due to the primary disease rather than to infection.
Insights
Brain and meningeal involvement in Wegener's granulomatosis (WG) is uncommon. This case study highlights WG with pial enhancement and perivascular space involvement, confirmed as granulomatous infiltration, not infection.
Area of Science:
- Neurology
- Pathology
- Rheumatology
Background:
- Wegener's granulomatosis (WG), a rare autoimmune vasculitis, infrequently affects the central nervous system.
- Meningeal involvement in WG typically presents as dural enhancement on imaging, attributed to granulomatous infiltration.
Observation:
- This report details a rare case of WG presenting with unusual pial enhancement and perivascular space involvement on magnetic resonance imaging (MRI).
Findings:
- Pathological examination confirmed granulomatous infiltration within the pia mater and perivascular spaces.
- The observed infiltration was definitively linked to the primary disease (WG) and not an infectious process.
Implications:
- This case expands the understanding of neuro-WG, demonstrating WG's potential to infiltrate deeper meningeal layers and perivascular spaces.
- Highlights the importance of considering WG in the differential diagnosis of unexplained pial and perivascular enhancement in the brain, even in the absence of typical dural findings.