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(Stroke. 1995;26:492-495.)
© 1995 American Heart Association, Inc.

Articles

Sneddon's Syndrome With Granulomatous Leptomeningeal Infiltration

Richard L. Boortz-Marx, MD; H. Brent Clark, MD, PhD; Saul Taylor, MD; Kathleen M. Wesa, MD David C. Anderson, MD

From the Departments of Neurology (R.L.B.-M., D.C.A.), Radiology (S.T.), and Medicine (K.M.W.), Hennepin County Medical Center, and the Departments of Neurology (R.L.B.-M., H.B.C., D.C.A.) and Laboratory Medicine and Pathology (H.B.C.), University of Minnesota Medical School, Minneapolis.

Background There is limited neuropathologic information available from cases of Sneddon's syndrome in which strokes are associated with livedo reticularis. Pathogenesis of the syndrome is controversial, although current opinion favors a coagulopathy, often with antiphospholipid antibodies. We describe a case lacking antiphospholipid antibodies but having a granulomatous infiltration of the leptomeninges.

Case Description The patient presented at age 29 with stroke, livedo reticularis, essential hypertension, and Raynaud's phenomenon. Assessment uncovered no underlying disease, including absent antiphospholipid antibodies. A leptomeningeal biopsy showed granulomatous infiltration.

Conclusions The findings suggest that an inflammatory process plays a role in at least some cases of Sneddon's syndrome.

Key Words: skin disease • Sneddon's syndrome • vasculitis • young adults

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