Clinical Presentation and Management of Idiopathic Intracranial Hypertension (Pseudotumor Cerebri)
Weight loss of 5-15% and acetazolamide therapy are the first-line treatments for idiopathic intracranial hypertension, with surgical intervention indicated when vision is at imminent risk. 1
Clinical Presentation
Cardinal Features
- Headache (progressively more severe and frequent)
- Papilloedema
- Visual disturbances:
- Transient visual obscurations (unilateral or bilateral darkening of vision lasting seconds)
- Visual blurring
- Visual field defects
- Visual acuity loss (in advanced cases)
- Pulsatile tinnitus
- Horizontal diplopia (typically from sixth nerve palsy)
Associated Symptoms
- Dizziness
- Neck pain
- Back pain
- Cognitive disturbances
- Radicular pain
Neuroimaging Features 2
- Empty or partially empty sella
- Increased optic nerve tortuosity
- Enlarged optic nerve sheath
- Flattened posterior globe/sclera
- Intraocular protrusion of optic nerve head
- Transverse sinus stenosis
Management Algorithm
1. Initial Assessment and Diagnosis
- Brain MRI with venography to rule out secondary causes
- Lumbar puncture with opening pressure measurement (elevated)
- Complete ophthalmological evaluation (visual acuity, visual fields, papilloedema grading)
2. Risk Stratification
- Fulminant IIH: Imminent risk of visual loss
- Typical IIH: Woman of reproductive age with BMI ≥30 kg/m²
- Atypical IIH: Non-female, non-reproductive age, BMI <30 kg/m²
3. First-Line Treatment 2, 1
- Weight loss program: Goal of 5-15% reduction in body weight
- Referral to structured weight management program
- Low-salt diet
- Acetazolamide: Starting at 250-500 mg twice daily
- Gradually increase based on clinical response and tolerability
- Maximum dose up to 4 g daily as tolerated
- Monitor for side effects: paresthesia, dysgeusia, nausea, fatigue, renal stones
4. Alternative Medical Treatments 1
- Topiramate: When acetazolamide is not tolerated
- Start at 25 mg daily
- Weekly escalation to 50 mg twice daily
- Dual benefit: carbonic anhydrase inhibition and appetite suppression
- Furosemide: As second-line agent when acetazolamide is insufficient
5. Headache Management 1
- Acute treatment:
- NSAIDs or paracetamol
- Indomethacin (may have additional benefit due to ICP reduction)
- Triptans for migraine-like attacks (limited to 2 days/week)
- Preventive treatment:
- Migraine preventatives (candesartan, venlafaxine) for coexisting chronic migraine
- Avoid opioids
6. Surgical Interventions for Vision Preservation 2, 1
- Indications: Deteriorating visual function despite maximal medical therapy or severe visual loss at diagnosis
- Temporizing measure: Lumbar drain to protect vision while planning definitive surgery
- Preferred procedures:
- Ventriculoperitoneal (VP) shunt: First choice due to lower revision rates
- Lumboperitoneal shunt: Alternative option
- Optic nerve sheath fenestration: Particularly for asymmetric papilloedema
- Venous sinus stenting: Investigational treatment requiring documented pressure gradient >8 mmHg
7. Management of Acute Exacerbation with CSF Shunt in Place 2
- Assess for signs of CNS infection
- Evaluate papilloedema and visual function
- Consider shunt tap or lumbar puncture
- Investigate for low pressure symptoms
- Consider shunt revision if vision deteriorating
8. Follow-up and Monitoring 1
- Regular ophthalmologic evaluations:
- Severe papilloedema: Every 1-3 months
- Moderate papilloedema: Every 3-4 months
- Mild papilloedema: Every 6 months
- Adjust intervals based on clinical course
Important Considerations
Medication Side Effects
- Acetazolamide: Paresthesia, dysgeusia, nausea, fatigue, renal stones
- Topiramate: Cognitive slowing, paresthesia, weight loss, kidney stones
- Corticosteroids: Not recommended for long-term use due to side effects
Surgical Complications
- CSF shunts: Infection, obstruction, over-drainage, need for revisions
- Optic nerve sheath fenestration: Visual loss, diplopia, infection
Bariatric Surgery
For severely obese patients with IIH, bariatric surgery may be considered for long-term management, but is not useful as an acute intervention 3.
Prognosis
The main morbidity of IIH is visual loss, which is present in most patients but can usually be reversed if recognized early and treated appropriately. Maintained weight loss is difficult to achieve but essential for disease remission 2.