Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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March 10, 2016
Sneddon Syndrome
- Background:
- Sneddon syndrome (SS) is a rare, non-inflammatory, thrombotic vasculopathy affecting the small- and medium-sized arterial vessels of the brain and skin.
- Predominantly affects women in early middle-age and is characterized by the occurrence of cerebrovascular disease associated with livedo racemosa.
- Clinical Presentation:
- Intracranial and intraventricular haemorrhage are unusual presentations in SS.
- Usually associated (40–50%) with antiphospholipid antibodies
- Key Diagnostic Features:
- The main diagnostic criteria are generalized livedo racemosa with typical histopathologic findings on skin biopsy and focal neurologic deficits.
- Ischemic lesions on MRI
- DSA:
- Stenosis and/or occlusion of intracranial distal vessels with a dense pattern of vascular dilatation with transdural anastomoses involving the lenticulostriate arteries
- Large network of fine collateral vessels ("pseudoangiomatosis")
- The large arteries of the circle of Willis are not involved.
- Differential Diagnosis:
- Divry-Van Bogaert syndrome
- Cerebral thromoangiitis obliterans (Buerger disease)
- Reversible cerebral vasoconstriction syndrome
- Treatment:
- Long-term anticoagulation has been recommended for cerebral ischemic events, but the optimal management is still unknown. There are controversial results regarding effects of immunomodulatory agents.
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