A Case Report of Pontine Infarction as an Initial Manifestation of Systemic Lupus Erythematosus: Diagnostic Clues from MRI and Digital Subtraction Angiography

Abstract

Brainstem infarction due to vertebrobasilar insufficiency is a rare initial presentation of systemic lupus erythematosus (SLE) patients and small-vessel dissection as the direct cause of infarction has not been reported. We report the case of a 20-year-old female with acute infarction on the right side of the pons due to a small artery (pontine perforator) dissection, identified on digital subtraction angiography and high-resolution vessel wall MRI (vwMRI). She was diagnosed with SLE based on the presence of neurologic disorders and relevant laboratory findings. The pontine perforator-dissecting aneurysm had occluded and the right distal vertebral artery had resolved on subsequent vwMRI. She had a modified Rankin Scale (mRS) score of 1 at discharge with mild symptom improvement, and exhibited no further aggravation of symptoms at 3 or 12 months, maintaining an mRS score of 1.

Keywords: Digital Subtraction Angiography; Dissecting Aneurysm; Magnetic Resonance Imaging; Stroke; Systemic Lupus Erythematosus.

Fig. 1. Pontine Infraction as an initial manifestation of systemic lupus erythematosus in a 20-year-old female.

A. Initial MRI. DWI (left and middle) and B0 image of DWI sequence (right) depict an acute infarction (black arrows) and an old lacunar infarction (white arrow) in the right pons. B. Digital subtraction angiography images (left and middle) reveal the dilated vascular structure (black arrows) on the right side of the basilar artery. Time-of-flight MR angiography (right) reveals two hemispherical aneurysms in the right cavernous internal carotid artery and distal vertebral artery (white arrows). C. DWI and vwMRI at day 1 follow-up. DWI (left end) reveals the increased extent of acute infarction in the right pons. Proton density vwMRI images (right series) confirm the dilated vascular structure's (black arrows) origin from the basilar artery (white arrows), that is, the pontine perforator. D. Pre and post-contrast MRI images at day 1 follow-up reveal intramural hematoma (double arrow) and wall enhancement in the dissecting aneurysm (arrow) (left two). In the dissecting aneurysm of the right distal vertebral artery, there is a corresponding intimal flap (arrow) on the pre-contrast T1 vwMRI and wall enhancement (double arrow) on the contrast-enhanced T1 vwMRI (right two). DWI = diffusion-weighted imaging, vwMRI = vessel wall MRI E. The vwMRI at day 9 follow-up depicts occlusion of the dissecting aneurysm. Proton density image vwMRI depicts lumen occlusion (left, arrow) that is observable as high signal intensity (thrombus) in the lumen of the aneurysm on pre-contrast T1 vwMRI (right, arrow). F. The vwMRI and time-of-flight MR angiography on day 9 follow-up depict decreased wall enhancement and normalized diameter of the right distal vertebral artery dissection (thin arrows); however, the aneurysm in the right cavernous internal carotid artery is still visible (thick arrows). vwMRI = vessel wall MRI