What is the best course of treatment for a patient with a history of pseudotumor cerebri (idiopathic intracranial hypertension) presenting with fever, persistent vomiting, headache, and congestion?

Management of Fever, Vomiting, Headache, and Congestion in a Patient with History of Pseudotumor Cerebri

This patient requires urgent evaluation to distinguish between a benign viral illness and recurrent intracranial hypertension, with immediate ophthalmologic examination (fundoscopy) and neuroimaging if any red flags are present.

Immediate Assessment Priority

Perform fundoscopy immediately to assess for papilledema, as persistent headache and vomiting in a patient with pseudotumor cerebri history mandates evaluation for recurrent intracranial hypertension 1, 2. The American Academy of Neurology recommends that in the presence of persistent headache or vomiting, an immediate work-up including fundoscopy should be performed 3.

Key Clinical Red Flags to Assess

• Visual symptoms: Blurred vision, transient visual obscurations, visual field defects, or diplopia suggest active intracranial hypertension 3, 1
• Neurologic examination: Look specifically for sixth nerve palsy (the only acceptable focal finding in IIH), altered mental status, or other focal deficits that would suggest alternative diagnoses 2, 4
• Papilledema grade: Document presence and severity, as this drives urgency of intervention 1, 2
• Fever pattern: True fever with congestion suggests concurrent infectious process rather than IIH recurrence 3

Diagnostic Approach

If Papilledema is Present or Neurologic Examination is Abnormal

Obtain MRI of the brain and orbits with and without contrast immediately 3, 2. MRI is more sensitive than CT for detecting secondary signs of increased intracranial pressure including empty sella, dilated optic sheaths, tortuous optic nerves, and flattening of posterior globes 3. Add MR venography to evaluate for cerebral venous sinus thrombosis, which can mimic or complicate pseudotumor cerebri 2, 5.

If No Papilledema and Normal Neurologic Exam

The constellation of fever and congestion suggests a viral upper respiratory infection is the primary process. However, maintain high suspicion and lower threshold for imaging given the pseudotumor cerebri history 3. Treat symptomatically but arrange close ophthalmologic follow-up within 1-3 days if headache persists 1.

Treatment Algorithm Based on Findings

For Confirmed Recurrent Intracranial Hypertension

Weight loss remains the only disease-modifying treatment and should be emphasized regardless of other interventions initiated 1, 2. The American Academy of Neurology recommends a weight loss program with low-salt diet for all overweight IIH patients, with a goal of 5-10% weight loss 1, 4.

Medical Management

• Acetazolamide is first-line medical therapy for symptomatic patients or those with evidence of visual loss 1, 2
• Start with appropriate dosing and gradually increase as needed and tolerated 2
• Avoid steroids as primary treatment, as they can worsen the condition and promote weight gain 1

Urgent Surgical Intervention Indications

If there is evidence of declining visual function or severe visual loss at presentation, urgent surgical treatment is required to preserve vision 1. Options include:

• Ventriculoperitoneal shunt is the preferred CSF diversion procedure due to lower reported revision rates 1, 2
• Optic nerve sheath fenestration is effective and safe, particularly when performed by experienced clinicians 2
• A temporizing lumbar drain may be used while planning definitive surgical intervention 1

For Presumed Viral Illness Without IIH Recurrence

• Symptomatic treatment for upper respiratory infection
• Ensure adequate hydration given vomiting
• Avoid medications that might exacerbate IIH: tetracyclines, vitamin A, retinoids, steroids, growth hormone, thyroxine, and lithium 2
• Close monitoring with repeat fundoscopy if symptoms persist beyond expected viral course

Critical Pitfalls to Avoid

Do not perform serial lumbar punctures for management, as they are not recommended for IIH treatment 2. Do not assume all headaches in IIH patients are from elevated pressure—patients with IIH often develop migrainous headaches superimposed on headaches from raised intracranial pressure, requiring specific treatment approaches 2.

Asymptomatic patients at initial presentation may remain asymptomatic during recurrence, so objective ophthalmologic assessment is essential rather than relying on symptoms alone 2. Treatment failure rates are substantial, with worsening vision after stabilization occurring in 34% at 1 year and 45% at 3 years 1, 2.