Presentation
Presented with left hemiparesis. NIHSS was 14. Stroke protocol activated.
Patient Data
NECT shows some cortical atrophy, especially in the left occipital lobe, but is otherwise unremarkable.
Occlusion of the right anterior cerebral artery (ACA) is evident on CTA and CTP shows an area of ischemic penumbra in the vascular territory of the right ACA.
The preliminary angiogram of the right internal carotid artery confirmed occlusion of the A2 segment of the ACA. Several attempts at mechanical thrombectomy with different stentrievers were made without success. At image revision, a very fine irregularity and tortuosity of the distal branches of the right ACA and middle cerebral artery (MCA) ("pseudoangiomatosis") was noted.
The patient underwent brain MRI a few days after the event: an acute infarction in the right ACA territory is noted, but also a smaller acute infarction in the left distal ACA territory, involving the other half of the corpus callosum and part of the mesial occipital cortex. In addition, multiple bilateral, cortical-subcortical, predominantly but not only supratentorial chronic infarctions are noted, with sparing of the periventricular white matter. Finally, there is severe cortical atrophy with no significant microbleeds at T2* gradient echo imaging.
The patient had a diffuse cutaneous rash which was noted only after the very acute phase. It was deemed compatible with livedo reticularis and a skin biopsy was performed.
The papillary and reticular dermis shows a proliferation of thin-walled small-caliber vessels with hints of hyperplasia of the endothelial lining and an ectatic lumen. Traces of eosinophilic fibrinoid material can be recognized within the lumen. The lymphocytic inflammation is distributed around the adnexa and the vessels.
The whole picture was considered ascribable to Sneddon Syndrome
Case Discussion
Sneddon syndrome is a clinical diagnosis with a fairly characteristic skin rash, MRI findings and catheter angiography appearance. In this case, given the very acute presentation, the livedo reticularis and the "pseudoangiomatosis" on DSA were not noted. However, during the following few days, the patient was thoroughly studied. Of note, mechanical thrombectomy was unsuccessful even after several attempts with different devices, possibly because of the underlying pathology.
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