Granulomatous vasculitis

Case contributed by Rajesh S

Presentation

Right motor and sensory cortical epilepsy since 1 day

Patient Data

Age: 40 years
Gender: Male

Initial MRI

mri

Diffuse gyral and juxta cortical signal changes with altered diffusivity (better characterized on ADC images with reduced signal) with associated subcortical FLAIR hypo-intensity and decreased perfusion signal on ASL are noted involving bilateral frontal parenchyma.

SWI shows few micro and macro hemorrhages with venous congestion in the form of prominent frontal veins in bilateral frontal parenchyma. Prominent frontal veins are also noted on post-contrast images draining into deep venous system through prominent peri-medullary and ependymal veins.

Thick patchy to linear enhancement is noted involving bilateral frontal parenchyma. Pachymeningeal enhancement is seen along the interhemispheric fissure.

Few frontal parenchymal areas of patchy enhancement with pachy-meningeal thickening and enhancement noted along falx cerebri.

Prominent lacunes are noted with non-specific juxta cortical WM changes.

Impression:

In view of multifocal bleeds and pachy-meningeal involvement possibility of vasculitis including granulomatous causes (venous congestion and prominent peri-medullary veins are often seen in neuro-sarcoidosis) was considered.

Suggested DSA for further imaging evaluation and CSF correlation to exclude granulomatous vasculitis

dsa

Short segment narrowing of the superior sagittal sinus with delayed venous drainage of frontal cortical veins seen. No evidence of significant arterial stenosis or irregular narrowing was noted (not shown here). 

Followup MRI after few months

mri

Interval increase in numerous foci of blooming noted in bilateral frontal lobes s/o sub-acute to chronic bleeds.

A well-defined T1 and T2 hyperintense foci with blooming and hemosiderin rim measuring 1.1 x 0.7 cm is noted in the right frontal parenchyma, likely subacute hematoma.

Prominent frontal peri-medullary veins draining into the deep venous system through ependymal veins is re-demonstrated on SWI and post-contrast images.

Thick patchy to linear enhancement is noted involving bilateral frontal parenchyma and pachymeningeal enhancement is seen along the interhemispheric fissure. Sulcal meningeal/subpial enhancement seen in bilateral frontal and occipital regions(left > right).

Prominent lacunes are noted with non-specific juxta cortical WM changes.

Diffuse subcortical FLAIR hypointensity was noted in the bilateral frontal parenchyma.

Impression:

In view of interval increase in hemorrhagic components and persisting pachymeningitis,  granulomatous vasculitis with disease progression was considered. 

Post OP MRI

mri

After counseling and informed consent, biopsy was performed. 

Post-operative changes are noted along bi-frontal convexities with resection cavity and hemorrhagic signal changes.

Multifocal microbleeds are re-demonstrated. Few areas of interval prominent blooming are noted predominantly involving frontal and basifrontal regions.

Interval decrease in pachymeningeal enhancement was noted in bilateral frontal parenchyma with persistent enhancement along the falx.

CSF & HPE

pathology

CSF showed lymphocytic pleocytosis and elevated protein. HPE from bi-frontal regions confirmed granulomatous vasculitis.

Case Discussion

This 40-year-old male was admitted with refractory focal seizures. MRI brain showed bifrontal hemorrhages with prominent veins extending to adjacent regions with pachymeningeal involvement suggesting the possibility of underlying granulomatous vasculitis.

Lymphocytic pleocytosis on CSF with DSA and MRI features further indicated the same and biopsy was suggested to confirm the diagnosis.

He was given IV methylprednisolone for 5 days. In addition, rituximab 1 gram was also given for long term management. Superior sagittal sinus stenosis could be in relation to chronic venous thrombosis which could have happened due to sarcoidosis.

Follow up MRI after 15 days showed a decrease in pachymeningeal enhancement.

Acknowledgements:

  • Dr. Shriram Varadharajan DM, Dr.Meena Nedunchelian DNB (Neuroradiology),
  • Dr. Rajesh Shanker Iyer DM (Neurology),
  • Dr. Suresh Jayabalan MCh (Neurosurgery) 
  • Dr. Sangita S Mehta MD, Dr.Lavanya MD (Pathology)
  • Interventional radiology (IR) team under Dr. Mathew Cherian, MD
  • Kovai Medical Center & Hospital, Coimbatore, India.

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