Article Text

Unusual association of diseases/symptoms
Wegener’s granulomatosis confined to nervous system
  1. M Lucchetta1,
  2. M Favero2,
  3. F Schiavon2,
  4. A Cagnin1,
  5. R Manara3,
  6. A Rotilio1,
  7. E Orvieto4,
  8. C Giannini5,
  9. C Briani1
  1. 1
    University of Padova, Neurosciences, Via Giustiniani 5, Padova, Italy, 35128
  2. 2
    University of Padova, Rheumatology, Via Giustiniani 2, Padova, Italy, 35128
  3. 3
    University of Padova, Neuroradiology, Via Giustiniani 5, Padova, Italy, 35128
  4. 4
    University of Padova, Pathology, Via Gabelli, Padova, Italy, 35100
  5. 5
    Mayo Clinic College of Medicine, Laboratory Medicine and Pathology, Rochester Minnesota, USA
  1. chiara.briani{at}unipd.it

Summary

Wegener’s granulomatosis (WG) is a multisystemic necrotising granulomatous vasculitis of small and medium sized vessels, that primarily involves the upper and lower respiratory tracts, lung tissues and kidneys. Serum antineutrophil cytoplasmic antibodies (ANCA) are a sensitive and specific marker of WG. Whereas the peripheral nervous system is often involved in WG, central nervous system manifestations are reported only in 2–8%, and are rarely present at onset. We report on a patient with atypical neurological presentation of ANCA negative WG in whom the diagnosis was made only after a meningeal biopsy.

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Footnotes

  • Competing interests: none.

  • Patient consent: Patient/guardian consent was obtained for publication