Freiberg's Infarction as the First Clinical Presentation of Sneddon Syndrome

Area of Science:

• Neurology
• Vascular Medicine
• Pediatric Neurology

Background:

• Sneddon syndrome is a rare, non-inflammatory vasculopathy typically affecting adults, characterized by livedo racemosa and recurrent ischemic strokes.
• Neurologic manifestations include migraines and cognitive decline, often associated with systemic lupus erythematosus or antiphospholipid antibodies, though autoimmune workup can be negative.
• Optimal treatment remains undefined, posing challenges in patient management.

Observation:

• A case report of an 18-year-old female presenting with acute expressive aphasia and evidence of multiple ischemic strokes on MRI.
• The patient had a 3-year history of livedo racemosa and migraines, with no identified autoimmune or cardioembolic stroke causes.
• Notably, she had a prior diagnosis of Freiberg disease (avascular necrosis of the second metatarsal head), identified five years earlier.

Findings:

• The patient was diagnosed with Sneddon syndrome based on livedo racemosa and recurrent ischemic strokes.
• This represents the first documented association between Freiberg disease and Sneddon syndrome.
• The findings suggest Freiberg disease may be an early, preceding symptom of Sneddon syndrome.

Implications:

• This case highlights a rare cause of stroke in the pediatric population and introduces avascular necrosis as a potential early manifestation of Sneddon syndrome.
• Recognizing precursor symptoms like Freiberg disease years before stroke onset could enable earlier diagnosis and primary stroke prevention strategies.
• Increased awareness of Sneddon syndrome's varied presentations is crucial for timely intervention, especially in younger patients.