Sneddon Syndrome

Sneddon Syndrome is a rare, progressive condition that combines recurring ischaemic strokes with distinctive skin changes known as livedo reticularis. Most commonly affecting young to middle-aged adults, it is considered a non-inflammatory vasculopathy — a disease of small and medium-sized blood vessels that may severely impact cerebral circulation.

At The Royal Buckinghamshire Hospital, we support patients living with the neurological consequences of Sneddon Syndrome, particularly those recovering from stroke. Our experienced neurorehabilitation team offers consultant-led, personalised programmes to help individuals manage complex, long-term neurological symptoms.

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What Is Sneddon Syndrome?

Sneddon Syndrome is a cerebrocutaneous disorder, meaning it involves both the brain and the skin. The condition is characterised by:

• Recurrent strokes or transient ischaemic attacks (TIAs)
• Livedo reticularis – a purplish, net-like discolouration of the skin, often on the limbs or trunk
• Vascular abnormalities in the small and medium-sized arteries, especially those supplying the brain

Sneddon Syndrome is often misdiagnosed or delayed in diagnosis, as early symptoms may be subtle or attributed to other conditions.

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Causes and Risk Factors

The exact cause is unknown, but there are two main forms:

• Primary (idiopathic) Sneddon Syndrome – no underlying autoimmune cause
• Secondary Sneddon Syndrome – associated with antiphospholipid syndrome or systemic lupus erythematosus (SLE)

Risk factors may include:

• Autoimmune disorders
• Antiphospholipid antibodies
• Previous unexplained stroke or TIA in young adults
• Family history of early-onset vascular disease

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Symptoms

Neurological symptoms:

• Recurrent TIAs or ischaemic strokes
• Cognitive decline or memory problems
• Headaches
• Seizures
• Dizziness, visual disturbances, or speech issues
• Progressive vascular dementia in long-term cases

Skin symptoms:

• Livedo reticularis – lace-like, bluish-purple skin discolouration
• May be persistent and most visible in cold temperatures
• Usually appears before or during early stroke events

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Stroke Risk

Sneddon Syndrome is one of the rare non-atherosclerotic causes of stroke, especially in younger individuals. Stroke risk arises from:

• Narrowing or occlusion of small cerebral arteries
• Hypercoagulability (clotting tendency) in those with antiphospholipid syndrome
• Arterial damage leading to recurrent infarcts

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Diagnosis

Diagnosis can be complex and may require input from multiple specialists. Investigations include:

• Brain MRI or CT – showing past stroke damage
• Skin biopsy of affected areas
• Cerebral angiography
• Blood tests – including antiphospholipid antibody screening
• Neuropsychological assessment – to monitor cognitive changes

Due to the progressive nature of the disease, early recognition and rehabilitation planning are essential.

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Treatment & Management

There is no cure for Sneddon Syndrome, but treatment is focused on preventing strokes and managing symptoms. This may include:

• Antiplatelet medication (e.g. aspirin) or anticoagulants in cases with antiphospholipid antibodies
• Blood pressure and lipid control
• Anti-seizure medication, if seizures are present
• Neurorehabilitation for stroke recovery and cognitive decline
• Psychological support for emotional and behavioural changes

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Neurological Rehabilitation at Royal Bucks

If you have experienced a stroke or progressive cognitive decline due to Sneddon Syndrome, we offer:

• Consultant-led rehabilitation programmes
• Neurological physiotherapy to support mobility
• Occupational therapy to enhance daily function
• Neuropsychology and cognitive training
• Fatigue and pain management