Wegener's granulomatosis of the paranasal sinuses with orbital and central nervous system involvement-diagnostic imaging

Acta Neurol Belg. 2011 Sep;111(3):241-4.


Wegener's granulomatosis (WG) is a systemic vasculitis that can affect any organic system, but primarily involves the upper and lower respiratory tracts and the kidneys. WG relatively frequently affects the nervous system (in 30-50%), usually in the form of peripheral or cranial neuropathy. Involvement of the brain is reported in a very small percentage of patients (2%-8%). Three major mechanisms have been described as the cause of central nervous system (CNS) disease in WG: contiguous invasion of granuloma from extracranial sites, remote intracranial granuloma and CNS vasculitis. CNS involvement caused by contiguous invasion of granuloma from extracranial sites is the rarest. We report the case of a 37-year-old man with WG, manifested as a pulmonary and paranasal sinuses disease, with orbital and CNS involvement, caused by contiguous invasion from the paranasal sinuses. In this report, the rich spectrum of findings achieved by computed tomography and magnetic resonance are demonstrated. The importance of computed tomography in bony destruction PNS findings, and the importance of MR imaging in evaluation of the direct intracranial spread from nasal, paranasal and orbital disease are also emphasized.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Brain Diseases* / diagnostic imaging
  • Brain Diseases* / etiology
  • Brain Diseases* / pathology
  • Granulomatosis with Polyangiitis* / complications
  • Granulomatosis with Polyangiitis* / diagnostic imaging
  • Granulomatosis with Polyangiitis* / pathology
  • Humans
  • Magnetic Resonance Imaging*
  • Male
  • Orbital Diseases* / diagnostic imaging
  • Orbital Diseases* / etiology
  • Orbital Diseases* / pathology
  • Paranasal Sinus Diseases* / diagnostic imaging
  • Paranasal Sinus Diseases* / etiology
  • Paranasal Sinus Diseases* / pathology
  • Tomography, X-Ray Computed*