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Sneddon Syndrome: A Case Report Exploring the Current Challenges Faced with Diagnosis and Management
Jonathan Cleaver1, Mario Teo2, Shelley Renowden3
1Department of Neurology, Southmead Hospital, Bristol, United Kingdom.
Case Reports in Neurology
|January 23, 2020
Summary
Sneddon syndrome, a rare vasculopathy, can present atypically without livedo racemosa. This case highlights diagnostic vigilance and explores medical and neurosurgical management options for stroke complications.
Area of Science:
- Neurology
- Rheumatology
- Vascular Medicine
Background:
- Sneddon syndrome (SS) is a rare medium-vessel vasculopathy.
- It characteristically presents with livedo racemosa (LR) and stroke complications.
Observation:
- This case report details a female patient with acute stroke but initially no LR.
- Neuroimaging revealed multiple strokes, extensive vasculopathy, and fragile collateral vessels.
- The patient experienced progressive memory loss and transient ischemic attacks.
Findings:
- SS should be suspected in idiopathic medium-vessel vasculopathy, even without LR.
- Antiphospholipid antibody status is crucial for risk stratification of antithrombotic therapy.
- Hydroxychloroquine may offer anti-thrombotic benefits in SS.
Implications:
- This case underscores the importance of diagnostic vigilance for atypical Sneddon syndrome presentations.
- Management requires balancing antithrombotic therapies and considering neurosurgical interventions.
- Sneddon syndrome might represent a forme fruste of antiphospholipid syndrome or lupus erythematosus.
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