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Sneddon and antiphospholipid antibody syndromes causing bilateral thalamic infarction
1Department of Neurology, Vanderbilt University School of Medicine, Nashville, TN 37212-3375.
Pediatric Neurology
|May 1, 1994
Summary
Bilateral thalamic infarction in a child was caused by Sneddon syndrome and antiphospholipid antibody syndrome. Treatment involved intensive warfarin anticoagulation, adapted from adult protocols for this rare pediatric stroke.
Area of Science:
- Neurology
- Pediatric Neurology
- Vascular Neurology
Background:
- Sneddon syndrome is a rare, non-inflammatory occlusive hydrocephalus characterized by recurrent ischemic strokes and livedo reticularis.
- Antiphospholipid antibody syndrome (APS) is an autoimmune disorder associated with an increased risk of thrombosis.
- Pediatric stroke is a significant cause of long-term disability, with diverse underlying etiologies.
Observation:
- A pediatric patient presented with hypersomnolence, mood disturbance, and amnesia, indicative of bilateral thalamic infarction.
- Diagnostic workup revealed the co-occurrence of Sneddon syndrome and antiphospholipid antibody syndrome as the cause of the stroke.
- This case highlights an unusual presentation of these conditions in a pediatric patient.
Findings:
- Bilateral thalamic infarction in a child is a rare manifestation of combined Sneddon and antiphospholipid antibody syndromes.
- The clinical presentation included neurological deficits consistent with thalamic injury.
- Laboratory evaluations confirmed the presence of antiphospholipid antibodies and features of Sneddon syndrome.
Implications:
- This case underscores the importance of considering Sneddon and antiphospholipid antibody syndromes in pediatric patients with unexplained ischemic strokes, particularly those with thalamic involvement.
- Treatment strategies, including long-term, high-intensity warfarin anticoagulation, may be extrapolated from adult management protocols for APS and related thrombotic disorders.
- Further research is warranted to elucidate the specific pathophysiology and optimize management of Sneddon syndrome and APS in the pediatric population.