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Published on: September 20, 2018
[A childhood case of antiphospholipid syndrome]
I Chabchoub1, A Ben Thabet, I Maaloul
1Service de pédiatrie générale, hôpital Habib-Bourguiba, Sfax, Tunisie. chabchoubimen@yahoo.fr
Insights
Pediatric cerebral strokes may stem from antiphospholipid syndrome, a rare childhood condition. This diagnosis is considered when common causes are excluded, especially in cases with high anticardiolipin antibodies.
Area of Science:
- Pediatric Neurology
- Rheumatology
- Hematology
Background:
- Cerebral strokes in children can arise from various causes.
- Antiphospholipid syndrome (APS) is a rare but significant cause of stroke in pediatric populations.
- APS is more commonly associated with systemic lupus erythematosus in adults.
Observation:
- A 17-month-old girl presented with hemiplegia and facial paralysis following viral infections.
- Cerebral MRI revealed internal carotid artery thrombosis and Sylvian artery territory ischemia.
- Extensive testing ruled out common thrombophilias and autoimmune diseases like lupus.
Findings:
- High levels of IgM anticardiolipin antibodies were persistently detected.
- A diagnosis of primary antiphospholipid syndrome was established in the absence of other clear etiologies.
- The patient remained free of further thrombotic events on anticoagulant and antiplatelet therapy.
Implications:
- Antiphospholipid syndrome should be considered in the differential diagnosis of pediatric cerebral strokes.
- Early identification and management of APS can prevent recurrent thrombotic events in children.
- This case highlights the importance of considering rare autoimmune conditions in pediatric stroke workups.
Unlabelled:
Many conditions can lead to cerebral strokes in children. The antiphospholipid syndrome widely described in adults in association with systemic lupus erythematosus, is rare in childhood.
Case Report:
Two months after recovering from varicella and a few days after an episode of bronchitis, a 17-month-old girl developed left facial paralysis associated with right hemiplegia. Brain MRI and angio-scan showed thrombosis in the internal left carotid associated with ischemia in the superficial posterior territory of the left Sylvian artery. Echocardiography and hemoglobin electrophoresis were normal. Tests were negative for protein S, C and antithrombin III deficiencies and no resistance to activated protein C. IgM anticardiolipin antibodies were detected at high level (greater than 25IU/l) initially and six weeks later. In the absence of an evident etiology, mainly systemic lupus erythematosus (negative antinuclear antibodies), the diagnosis of primary antiphospholipid syndrome was retained. The girl was treated by heparin then by salicylate at antiaggregate doses associated with re-habilitation. Twelve months later, the patient had not developed any other thrombosis, in spite of a high level of anticardiolipin antibodies.
Conclusion:
In children with cerebral strokes, antiphospholipid syndrome must be discussed when the usual etiologies have been ruled out.
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