Systemic Lupus Erythematosus Cerebral Vasculitis

Posted By Taylor Schwartz
Systemic Lupus Erythematosus Cerebral Vasculitis

Case Presentation: 31-year-old female with Systemic Lupus Erythematosus presented to the ED after an unwitnessed seizure. Patient was found on the floor in her bathroom unresponsive. She remained altered in the ER with obtundation. Imaging studies including contrast brain MRI and formal cerebral angiogram were performed. Imaging Findings: MRI of the brain was markedly abnormal. T1 post contrast imaging demonstrate diffuse multifocal nodular enhancement, predominantly at the gray white junction of the cerebral and cerebellar hemispheres, mid-brain, pons and thalami. (Images 1-5). Fluid sensitive sequences demonstrate intense signal abnormalities mirroring the pathologic enhancement pattern. Cerebral angiogram demonstrates marked pruning of the distal arterial branches at the grey-white junction, with altered distal capillary phase. Abnormal beading and irregularity of terminal arteries is also shown indicating abnormalities of Lupus small vessel vasculitis. Additionally, truncated and missing vessels are seen throughout and can be appreciated in image 8. (Images 6-8) Teaching Point: Imaging of a clinically severe cerebral vasculitis in a patient with SLE can manifest in both explicit and more subtle ways. MRI is the modality of choice when assessing suspected cerebral vasculitis because of the direct abnormalities seen. The T1 post contrast images in this patient clearly demonstrates multifocal enhancement abnormalities. (Images 1-3) The abnormal enhancement pattern is magnified with fluid sensitive sequences. (Images 4-5). This MRI enhancement pattern, while dramatic, is classic for small vessel cerebral vasculitis. However, it is the cerebral angiogram which makes this case noteworthy. The angiogram demonstrates a paucity of complete and patent distal arterial branches, and shows a uniform lack of normal enhancement at the grey-white junction during the capillary phase. The truncated small vessels and irregular beading of arteries are other findings easily missed. (Images 6-8)

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