Idiopathic Intracranial Hypertension (Pseudotumor Cerebri)



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Idiopathic Intracranial Hypertension

(Pseudotumor Cerebri)



  • Cause unknown but many associated conditions.

  • Characterized by ICP & papilledema.




  • Incidence in general population: 1 per 100,000

Obese _ of child bearing age: 19 per 100,000


  • Symptoms: Headaches, TVO’s, blurred vision, diplopia, pulsatile tinnitus, neck pain.

Consciousness not altered.


  • Only neurological complication is visual loss due to papilledema.



Classification of IIH


  1. IIH due to unknown cause

Most common. No identifiable associated condition.

Criteria: a) Normal CT/MRI

b) LP shows normal contents and high pressure

c) Papilledema may or may not be present.

NB. No papilledema no threat to vision.





  1. Symptomatic IIH




Associated condition present.

Patients with viral, bacterial, fungal infections, tumors, inflammatory conditions that raise ICP do not have IIH.



Clinical Evaluation


  1. Perimetry: Disc related field defects.

Enlarged blind spot

Nasal inferior constriction

Arcuate defects

Generalized constriction


  1. RAPD




  1. Visual Acuity: Least sensitive clinical measure.

Causes of permanent visual loss include:


Optic nerve infarction

Macular changes

Subretinal hemorrhage

Ischemic and compressive damage to optic nerves




  1. Fundus photography



Treatment






Medical Treatment


  1. Acetazolamide 1000 to 2000 mg/day

No prospective randomized controlled clinical trials.


  1. Furosemide 40 to 160 mg/day




  1. Corticosteroids

Effective initially. Papilledema recurs.
Surgery for visual loss.

Optic nerve fenestration is the procedure of choice.
Corbett JJ, et al: Results of Optic Nerve Fenestration for Pseudotumor Cerebri. Arch Ophthalmol 1988;106:1391-97.

28 pts, 20_, 8_ with progressive VF defect, decreasing VA.

40 eyes.

Results: VF unchanged or improved in 80%

VA unchanged or improved in 85%

Papilledema resolved in 100%

53% showed improvement in fellow eye not operated on

68% H/A improved

88% TVO’s resolved

Does not seem to lower ICP


Complications: 16/40 tonic pupils and accommodative paresis.

1/40 retrobulbar hem with visual loss


Recommendations for surgery:

  1. Development of new VF defect.

  2. Enlargement of previously existing VF defect.

  3. Increasing RAPD.

  4. Visual loss.

Unilateral operation recommended unless severe bilateral visual loss or in patients with high surgical risk.




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