Sneddon's Syndrome Details

Expanded Description

Sneddon’s generally makes itself known through unusual neurological symptoms including TIA-like symptoms (sudden weakness of one or both sides, disorientation, difficulty speaking) or stroke at an early age. Its patients always have “livedo reticularis” (or “livedo racemosa”, the terminology varies), which is a purplish mottling on the skin that can worsen in cold.

Other Sneddon’s symptoms can include eye pain (which is sometimes very severe, causing nausea), strokes in the eye, memory or concentration problems, changes in personality or character, exercise intolerance, strokes at a young age with no apparent cause.

Sneddon’s is caused by a degenerative problem with the linings of the arteries, which generally leads first to livedo in the skin, and then, usually many years later, neurological difficulties. Arterial difficulties can occur anywhere in the body in Sneddon’s patients, though they will generally occur in the medium to small (or very small) vessels. As a result of compromised vessel lining, clotting is the primary concern with Sneddon’s, though plaque may also form (with or without high cholesterol), and malformations of the vessel lining may lead to occlusion.

Originally, it was thought that Sneddon’s was a form of Antiphospholipid Antibody Syndrome, but it has now been shown that less than half of patients with Sneddon’s test positive for this disease. As a result, Sneddon’s has been reclassified as a cerebrovascular disease (rather than autoimmune disease, like antiphospholipid antibody syndrome). While it seems that Sneddon’s may have inflammatory origins, the disease process is not inflammatory.

It is often very difficult for Sneddon’s patients to get a definitive diagnosis because of the lack of a clear test, and many spend years, or lifetimes, suffering with misdiagnoses (often “vasculitis”) treatments that worsen symptoms, and suggestions that the patient’s psychiatric state is the source of the problem. For the Sneddon’s patient, who cannot help but know that his or her neurological symptoms are severe and highly threatening, this can be emotionally debilitating.

Often when Sneddon’s seems likely doctors will try treatment with anticoagulant medications (Warfarin/Coumadin) and use the success of that treatment as a diagnostic tool. Sneddon’s patients gain the greatest relief on warfarin, with an INR between 3-4.

Prevalence

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Causes

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Disorder Symptoms

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Diagnosis

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Diagnostic Tests

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Disorder Treatments

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Prognosis

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