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Wegener granuloma in the fourth ventricle
Ansgar Berlis1, Franziska Petschner, Ingolf Carl Bötefür
1Department of Neuroradiology, University of Freiburg, Freiburg, Germany.
AJNR. American Journal of Neuroradiology
|March 15, 2003
Summary
A rare case of Wegener granulomatosis (now ANCA-associated vasculitis) affected the brain's fourth ventricle in a 57-year-old man. Steroid treatment initially reduced the mass, but the patient later died from pneumonia.
Area of Science:
- Neurology
- Rheumatology
- Pathology
Background:
- Wegener granulomatosis, now known as granulomatosis with polyangiitis (GPA), is a rare autoimmune disease.
- GPA typically affects the upper and lower respiratory tracts and kidneys.
- Central nervous system (CNS) involvement is uncommon but can occur.
Observation:
- A 57-year-old man with known GPA presented with new CNS symptoms including nausea and altered mental status.
- Brain MRI revealed a mass in the fourth ventricle.
- The mass showed a significant reduction in size following systemic steroid therapy.
Findings:
- Histopathology confirmed the fourth ventricular mass was a Wegener granuloma.
- Despite initial positive response to steroids, the patient succumbed to pneumonia.
Implications:
- This case highlights the potential for GPA to manifest as a CNS mass.
- It underscores the importance of considering GPA in patients with unexplained neurological symptoms and respiratory involvement.
- Early recognition and management of CNS GPA may be crucial, although outcomes can be severe.