Presentation
The patient presented with a disturbed level of consciousness.
Patient Data
There is a high signal intensity in the left frontal, parietal and temporal lobe on T2 and FLAIR imaging. This involves the deep and subcortical white matter. There are multiple
foci of hemorrhage as evidenced by the high T1 signal, and low T2 signal, with blooming on the GRE. There is a linear subcortical and ring-like enhancement on contrast administration.
There is an abnormal FLAIR signal within bilateral basal ganglia, left greater than right, with micro hemorrhages as evident by the blooming on the GRE sequence.
Linear low signal intensity on GRE along the right cerebellar folia is consistent with hemosiderin deposition and chronic bleeding.
Linear high signal intensity at the left convexity surface sulci on FLAIR, with post-contrast enhancement suggestive of leptomeningeal collaterals (ivy sign).
The differential diagnosis is wide and includes:
acute hemorrhagic leukoencephalitis,
viral encephalitis,
CNS vasculitis,
tumor (hemorrhagic metastasis).
The patient rapidly deteriorated and underwent a brain biopsy, which revealed multiple hemorrhagic infarctions caused by CNS vasculitis.
Case Discussion
The patient underwent several examinations to search for a source of the cerebral metastasis, all of which were negative. Laboratory evaluation for secondary vasculitis was negative. The patient deteriorated and underwent a brain biopsy.
Primary CNS angiitis presents with a variety of non-specific imaging abnormalities, with ischemic infarctions accounting for the majority of lesions (around 60% of cases).
The combination of multiple sites of intracerebral hemorrhage, basal ganglia micro hemorrhage, subarachnoid hemorrhage and meningeal enhancement with the confirmed absence of occult neoplasia or a potential systemic cause alludes to the diagnosis of primary CNS vasculitis in this instance as suggested by the brain biopsy.
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