A 34-year-old woman presented with acute onset left facial weakness and dysarthria. Examination showed diffuse livedo racemosa (figure, A). Brain MRI demonstrated right frontal acute stroke (figure, B), with numerous chronic ischemic lesions in the deep white matter (figure, C). CT angiogram revealed multiple irregularities along the cortical branches of intracranial vessels (figure, D and E). Skin biopsy was nonspecific.1 Extensive immunologic, hematologic, and infectious workup was unremarkable.2 The patient was diagnosed with Sneddon syndrome and discharged on aspirin.
(A) Skin examination shows network-like violaceous-erythematous patches in the legs consistent with livedo racemosa (arms and trunk are not shown). (B) Brain MRI shows restricted diffusion in the right frontal lobe (arrows) with corresponding reduced signal on apparent diffusion coefficient consistent with acute ischemic stroke (not shown). (C) Fluid attenuated inversion recovery sequence shows numerous chronic ischemic lesions in the deep white matter and cortical-subcortical regions of both cerebral hemispheres (arrow). (D and E) Brain CT angiogram shows multiple irregularities along the cortical branches of the right and left middle cerebral arteries, mainly at the M2 and M3 levels (arrows). Similar irregularities are present along the right posterior cerebral artery (not shown).
Sneddon syndrome is a noninflammatory arteriopathy presenting classic neurovascular and dermatological signs. Often associated with antiphospholipid syndrome or autoimmune disorders, its pathophysiology remains unknown.
Study funding
No targeted funding reported.
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Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Teaching slides: links.lww.com/WNL/A954.
- © 2019 American Academy of Neurology
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