Granulomatosis with polyangitis

Case contributed by Dr Eric F Greif

Presentation

Multiple episodes of nose bleeds along with severe recurrent headaches.

Patient Data

Age: 65
Gender: Female
CT

CT Brain

There is thin linear hyperdensity in the anterior interhemispheric fissure. There are adjacent bilateral  subcortical  foci of white matter hypodensities in the surrounding cingulate gyri, with partial sulcal effacement in the medial frontal lobes.

There is bilateral sphenoid and maxillary sinusitis.

Status post left canal wall up mastoidectomy.

MRI

MRI Brain

There is a 1.5 X 0.4 cm ovoid mass in the anterior interhemispheric fissure along the falx corresponding to the hyperdensity on the recent CT. The mass is low in signal intensity on T2 and FLAIR imaging and isointense to gray matter on T1-weighted imaging. The mass does not demonstrate blooming susceptibility artifact on GRE or restricted diffusion. After the administration of intravenous gadolinium there is peripheral enhancement of the anterior falx most pronounced around the ovoid mass with adjacent leptomeningeal enhancement over the frontal lobes, more pronounced on the left. There is abnormal hyperintense signal intensity on FLAIR in the adjacent parasagittal frontal lobes, more pronounced on the right.

There are minimal scattered foci of white matter signal hyperintensity on T2 and FLAIR imaging.

There has been a prior partial left mastoidectomy. There is opacification of the right mastoid air cells. The maxillary sinuses are hypoplastic and opacified. There is near total opacification of the posterior ethmoid air cells.

CT

CT Chest

There are thick rimmed cavitary lesions in bilateral upper and right lower lobes. The largest lesions measure 1.9-cm in the right lower lobe. Posterior right lower lobe cavitary lesions are surrounded by ground glass opacity.

Case Discussion

Ovoid nodule of T2 hypointensity and T1 isointensity in the anterior hemispheric fissure with peripheral enhancement, adjacent dural/leptomeningeal enhancement and adjacent parasagittal frontal lobe vasogenic edema. This finding corresponds with hyperdensity on a recent head CT. The findings are nonspecific and may represent intracranial involvement of patients recently diagnosed granulomatosis with polyangiitis (Wegener's granulomatosis) or CNS lymphoma. The MR imaging appearance is atypical for acute subdural hematoma.

The collection of findings: Cavitary lung lesion and sinusitis should raise concern for granulomatosis with polyangiitis. A brain biopsy was performed to further evaluate the extra-axial mass.

Pathology:

Brain, Biopsy: Designated falcine; left frontal craniotomy, biopsy under stealth guidance:

Necrotizing granulomata.

Lung biopsy: 

Compatible with ANCA positive vasculitis (Wegener's granulomatosis) with necrotizing granulomas.

Findings are compatible with CNS involvement by granulomatosis with polyangiitis.

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Case information

rID: 35506
Case created: 8th Apr 2015
Last edited: 30th Jun 2016
Inclusion in quiz mode: Included

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