What is the recommended treatment for a patient presenting with symptoms suggestive of cerebral venous thrombosis?

Treatment of Cerebral Venous Thrombosis

The recommended first-line treatment for patients with cerebral venous thrombosis (CVT) is immediate anticoagulation with either low-molecular-weight heparin (LMWH) or intravenous unfractionated heparin (UFH), even in the presence of hemorrhagic lesions. 1, 2

Initial Diagnostic Approach

• Confirm diagnosis with appropriate imaging:
  • MRI with MR venography is the preferred initial imaging modality 1
  • CT venography can be used if MRI is not readily available 1
  • For patients with high clinical suspicion of CVT, perform same-day imaging based on location of symptoms 3

Initial Treatment Algorithm

1. Immediate Anticoagulation:

   • Start parenteral anticoagulation as soon as CVT is diagnosed 1, 2
   • LMWH is preferred over UFH due to superior efficacy 2
     • Enoxaparin: 1.0 mg/kg twice daily or 1.5 mg/kg once daily 2
     • Dalteparin: 200 U/kg once daily 2
   • UFH is an appropriate alternative when LMWH is contraindicated, unavailable, in cases of severe renal failure, or when thrombolytic therapy may be needed 2, 4
     • Initial bolus of 5000 IU, followed by continuous infusion of approximately 30,000 IU over 24 hours, adjusted to maintain aPTT at 1.5-2.5 times baseline 2

2. Important Clinical Consideration:

   • The presence of intracerebral hemorrhage related to CVT is NOT a contraindication to anticoagulation 1, 2, 5
   • Some patients with cerebral venous sinus thrombosis may experience rapid deterioration after appearing clinically well 3

Special Circumstances

For Vaccine-Induced Immune Thrombocytopenia and Thrombosis (VITT) with CVT:

• Immediately administer 1 g/kg intravenous immunoglobulin 3
• Use non-heparin based anticoagulants (direct oral anticoagulants, fondaparinux, or danaparoid sodium) 3
• Monitor fibrinogen levels and maintain at least 1.5 g/L 3

For CVT associated with Behçet's syndrome:

• Administer high-dose glucocorticoids followed by tapering 3, 2
• Add anticoagulants for a short duration 3, 2

Transition to Oral Anticoagulation

• Begin oral anticoagulation early (same day as parenteral therapy is started) 3, 2
• Continue parenteral anticoagulation for a minimum of 5 days and until INR ≥2.0 for at least 24 hours 3, 2
• For patients treated with vitamin K antagonists (VKA), maintain INR between 2.0-3.0 (target 2.5) 2

Duration of Anticoagulation

• Minimum duration of 3 months for all patients with CVT 2
• For CVT secondary to a transient risk factor: 3-6 months 1, 2, 5
• For idiopathic CVT or mild hereditary thrombophilia: 6-12 months 5
• Consider indefinite (lifelong) anticoagulation for:
  • Severe thrombophilia 1
  • Recurrent thrombosis 1, 5
  • Antiphospholipid syndrome 2

Follow-up Management

• Admit all patients to a stroke unit for close monitoring and specialized care 1
• Perform regular neurological assessments to detect clinical deterioration 2
• Consider follow-up CT venography or MR venography at 3-6 months to assess recanalization 2

Potential Pitfalls and Caveats

• Delayed diagnosis is common due to the wide spectrum of clinical presentations 6, 5
• D-dimer may be elevated in most patients but normal D-dimers do not rule out CVT 6
• Some patients (approximately 5%) may have normal platelet counts at presentation that subsequently fall below normal; repeat platelet count after 2-3 days if clinical suspicion remains high 3
• When starting anticoagulation in patients with CVT and subarachnoid hemorrhage, it may be prudent to repeat CT after at least 24 hours from symptom onset to confirm regression or non-progression of hemorrhage 7