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Cerebral vein thrombosis in childhood systemic lupus erythematosus
1Department of Pediatrics, Hospital for Sick Children, Toronto, Canada.
Insights
Systemic lupus erythematosus (SLE) can cause cerebral vein thrombosis (CVT) presenting as severe headaches. Early diagnosis and treatment of CVT in SLE patients are crucial for preventing severe neurological damage.
Area of Science:
- Neurology
- Rheumatology
- Vascular Medicine
Background:
- Cerebral vein thrombosis (CVT) is a rare but serious condition.
- Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect various organs, including the brain.
- CVT is an uncommon neurological complication of SLE.
Observation:
- Three cases of adolescent girls with SLE presenting with severe, unremitting headaches are described.
- Clinical suspicion for CVT was high, but diagnosis was delayed in one patient, resulting in a cerebral infarct.
- In the other two patients, earlier diagnosis of CVT was achieved.
Findings:
- Cerebral vein thrombosis (CVT) was diagnosed in all three patients using non-invasive imaging techniques (CT and MRI).
- Early diagnosis and treatment in two patients appeared to prevent further neurological sequelae.
- All patients received anticoagulation therapy and management for their underlying SLE.
Implications:
- This case series highlights the importance of considering CVT in SLE patients presenting with severe headaches.
- Prompt neuroimaging and initiation of anticoagulation are critical for favorable outcomes in SLE-associated CVT.
- Long-term anticoagulation with warfarin appears effective in preventing recurrence of CVT in this patient population.
Abstract:
We describe three cases of cerebral vein thrombosis (CVT) in girls with systemic lupus erythematosus. Severe, persistent, unremitting headache was a common manifestation. In the first patient, although the clinical features were suggestive of CVT, the diagnosis was delayed and she had a significant cerebral infarct. In the other two patients the diagnosis was made earlier and led to more rapid treatment; the institution of early therapy may have prevented further sequelae. The CVT was diagnosed in all patients with a combination of computed tomography and magnetic resonance imaging studies without the need for angiography. All patients were treated for their underlying systemic lupus erythematosus and with anticoagulation. All are receiving long-term low doses of warfarin and have not had any recurrences.