Urgent Neuroimaging Required for Suspected Increased Intracranial Pressure
This patient requires immediate brain MRI with gadolinium to exclude life-threatening conditions including cerebral venous sinus thrombosis, hemorrhage, stroke, or mass lesions, as headache with vomiting upon waking represents a high-risk feature in SLE patients that mandates comprehensive evaluation beyond standard headache assessment. 1
Immediate Diagnostic Work-Up
Neuroimaging Priority
- Obtain brain MRI with gadolinium-enhanced T1 sequences and diffusion-weighted imaging (DWI) urgently to exclude stroke, hemorrhage, cerebral venous sinus thrombosis, or inflammatory lesions 1
- Look specifically for signs of increased intracranial pressure: diffuse brain edema, ventricular compression, or empty sella 2
- MRI may show periventricular and subcortical focal lesions (3-22 mm diameter) in SLE patients with neurological symptoms 3
CSF Analysis
- Perform lumbar puncture with opening pressure measurement after imaging excludes mass effect 1
- Opening pressure >250 mmH2O suggests increased intracranial pressure (IIH has been reported with pressures up to 350 mmH2O in SLE) 2
- Include cell count, protein, glucose, Gram stain, culture, and viral PCR (HSV, JC virus) to exclude CNS infection 1, 4
- Mild CSF abnormalities (elevated protein) occur in 40-50% of neuropsychiatric SLE but are non-specific 4
Laboratory Evaluation
- Check complement levels (C3, C4), anti-dsDNA antibodies, and antiphospholipid antibodies for prognostic information 1
- Critical caveat: Normal CBC, renal function, and liver enzymes do not exclude active CNS lupus, as neurological involvement can occur independently of systemic disease activity 1
Clinical Syndrome Recognition
Increased Intracranial Pressure in SLE
The pattern of morning headaches and vomiting after physical activity strongly suggests increased intracranial pressure, which occurs in approximately 1.5% of hospitalized SLE patients (far exceeding the general population prevalence of 1-19 per 100,000) 5
Key clinical features to assess:
- Papilledema on fundoscopic examination (present in most but not all cases) 2, 6
- Visual changes or diplopia from cranial nerve VI palsy 7
- Altered mental status or focal neurological signs 8
- Fever or meningismus suggesting infection 8
Differential Diagnosis Requiring Exclusion
- Cerebral venous sinus thrombosis (especially with antiphospholipid antibodies) 8
- Aseptic or septic meningitis 8
- Subarachnoid or intracerebral hemorrhage 8
- Brainstem stroke or structural lesions 9
Treatment Algorithm
If Increased Intracranial Pressure Confirmed
Initiate pulse intravenous methylprednisolone combined with intravenous cyclophosphamide for severe neuropsychiatric SLE manifestations, with expected response rates of 60-75% 9, 1
Adjunctive measures:
- Acetazolamide for ICP reduction (used in 9 of 10 patients in one series with good response) 5
- Osmotic diuretics (mannitol) for acute management 2
- Serial lumbar punctures if refractory 5
Expected Timeline
- Clinical improvement should occur within days to 3 weeks with appropriate immunosuppressive therapy 9, 1
- Neurological response should parallel improvement in systemic disease activity 9
Maintenance Therapy
Chronic immunosuppressive therapy is necessary given the 50-60% relapse rate during corticosteroid dose reduction 9
- Options include azathioprine, mycophenolate mofetil, or continued cyclophosphamide 9
If Antiphospholipid Antibodies Present
- Consider anticoagulation if thrombotic mechanism identified on imaging 1
- Two patients in one series had elevated antiphospholipid antibodies with thromboembolic events 5
Critical Pitfalls to Avoid
- Never attribute neurological symptoms to lupus without imaging and appropriate exclusion of infection, stroke, or hemorrhage 1
- Delayed treatment initiation beyond 2 weeks is associated with severe neurological deficits 1
- Do not assume headache is benign in SLE—this patient's symptom pattern (morning predominance, vomiting, post-exertional) demands urgent evaluation 8, 1
- Previous severe neuropsychiatric SLE manifestations confer at least a fivefold increased risk for subsequent neuropsychiatric events 1
- Some patients show chronic persistent intracranial hypertension with poor response to standard treatments, requiring prolonged management 6