CNS vasculitis

Case contributed by Dr Ernest Lekgabe

Presentation

Presented with left facial droop and left upper limb weakness

Patient Data

Age: 25
Gender: Male
CT

Hypoattenuation involving the right caudate and lentiform nucleus is favoured to represent a combination of chronic and subacute ischaemia.

CT

There is a focal stenosis of a left M2 superior division branch, as well as a more distal left inferior division M2/3 high grade stenosis/complete occlusion.

There are also high grade stenoses/occlusion of right M2 branches.

DSA (angiography)

Similar to CTA, there is a focal stenosis of a left M2 superior division branch, as well as a more distal left inferior division M2/3 occlusion. 

Occlusion of right M3/M4 branches of the inferior M2 MCA division.

MRI

Multiple infarcts of varying age in multiple vascular territories. Foci of SWI hypointensity in predominantly sulcal distribution, but with a lobulated/punctate morphology and conforming to the course of MCA branches in both Sylvian fissures, and the right superior cerebellar artery (which does not fill on TOF MRA). Irregularity of the M1 segment right MCA best appreciated on CT. Focal stenosis left MCA branch. Overall appearance suggestive of cerebral vasculitis with obliterative component.

MRI

There is widespread long segment concentric vessel wall thickening and hyperenhancement throughout the brain, involving particularly the M2, M3 and M4 MCA branches bilaterally, bilateral PCAs and right medial lenticulostriate vessels. Known focal stenosis of the superior M2 division of the left MCA. Occluded M3 branch of left inferior M2 division with focal T1 hyperintensity in the left sylvian fissure. Occluded M3 branch of the right inferior M2 division. Known subacute infarct in the right basal ganglia.

Conclusion:

Extensive bilateral hemisphere long segmental vessel wall thickening and hyperenhancement in keeping with vasculitis. Secondary and primary vasculitis both need to be considered.

Pathology

MICROSCOPIC DESCRIPTION:

Mild inflammatory changes in pia-arachnoid and cortical perivascular spaces

COMMENT:

The mild inflammatory changes seen in the cortical biopsies fall short of current criteria for the pathological diagnosis of isolated cerebral angiitis. However, the changes may be related to angiitis that is not sampled in the biopsies submitted.

Case Discussion

Diagnosis:

Most likely CNS vasculitis, based on clinical, radiological and histological findings.

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Case information

rID: 55715
Case created: 21st Sep 2017
Last edited: 5th Nov 2017
Inclusion in quiz mode: Included

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