Cerebral amyloid angiopathy related inflammation

Case contributed by Mohamed Ismail Saber Ismail
Diagnosis almost certain

Presentation

Acute confusion for 3 days. No previous relevant medical history.

Patient Data

Age: 80 years
Gender: Male

Bilateral areas of vasogenic brain edema in both temporal and parieto-occipital lobes.

Hemorrhagic focus noted in the posterior left temporal lobe.

Multiple Innumerable foci of SWI susceptibility artefact are seen through both cerebral hemispheres largely sparing basal ganglia, thalami and brain stem.

Extensive bilateral deep white matter and subcortical high T2 and FLAIR signal intensities representing areas of brain edema with slight posterior predominance with bilateral temporal lobe involvement.

No evidence of abnormal enhancement in the post-contrast sequence (images not included).

Case Discussion

The widespread distribution of the innumerable foci of susceptibility artefacts representing micro hemorrhages are in keeping with cerebral amyloid angiopathy.

Sparing of basal ganglia, thalami and brain stem is differentiating it from the appearance of chronic hypertensive angiopathy which has a predominance for the basal ganglia and thalami.

The presence of multiple regions of vasogenic edema (high signal in T2) involving the white matter with localized mass effect and most of the changes are confined to the subcortical white matter are in keeping with cerebral amyloid angiopathy related inflammation which also known as cerebral amyloid angiitis. No significant leptomeningeal enhancement could be seen in this case.

Although the posterior reversible encephalopathy syndrome ( PRES) is worth considering as a differential diagnosis as there are no acute risk factors, this is unlikely to be the case. 

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