Intracranial Hypertension

Description

Idiopathic Intracranial Hypertension is also called Pseudotumor cerebri syndrome and benign intracranial hypertension. It is a syndrome of raised intracranial pressure and papilledema, without mass lesion or enlarged ventricles. Prior to its diagnosis all other causes of raised intracranial pressure need to be excluded. It is more common in women (4-8 times more common) and is associated with obesity (over 93% of patients are obese). There has also been an association with a number of drugs including anti- acne medications and tetracycline antibiotics.

Symptoms

Symptoms may include

• Headache
• Visual loss (peripheral vision is lost first, central vision is not damaged until late in the illness)
• Tinnitus (whooshing sound in the head – often in time with the pulse)
• Nausea, vomiting

Diagnosis

Patients should undergo a complete neuro-ophthalmological examination by both a neurologist and ophthalmologists: including examination of the fundi looking for papilledema, and visual field testing.  If a patient is suspected of having IIH they need to undergo MRI brain and MR venography and lumbar punctures with CSF opening pressures measured and a CSF sample sent for analysis.

Treatment

• Medication e.g. acetazolamide
• Lumbar punctures to lower intracranial pressure

Older Surgical treatment options:

• CSF shunt diversion procedures
• Optical nerve sheath fenestration

Newer treatments/ recent research:

• Stenting of transverse sinus stenosis as treatment for IIH
  Reference: R.M. Ahmed, M. Wilkinson, G.D. Parker, M.J. Thurtell, J. Macdonald, P.J. McCluskey, R. Allan, V. Dunne, M. Hanlon, B.K. Owler and G.M. Halmagyi. Transverse Sinus Stenting for Idiopathic Intracranial Hypertension: A Review of 52 Patients and of Model Predictions. AJNR, 2011
• The association between IIH and obstructive sleep apnoea
  Reference: Thurtell M, Bruce BB, Rye DB, Newman NJ, Biousse V. The berlin questionnaire screens for obstructive sleep apnoea in idiopathic intracr5anial hypertension. J Neurophthal. 2011.

Further Reading

Patient Stories

• Idiopathic Intracranial Hypertension – Hayley’s Story

Journal Articles

1. Lueck CJ, McIlwaine GG. Interventions for idiopathic intracranial hypertension. Cochrane Database of Systematic Reviews 2005, Issue 3. Art. No.: CD003434. DOI: 10.1002/14651858.CD003434.pub2
2. Sinclair A. Idiopathic intracranial hypertension. Recent concepts and developments. ACNR. 2010; 3: 10-14.
3. Fraser C, Plant GT. The syndrome of pseudotumor cerebri and idiopathic intracranial hypertension. Current opinion in neurology. 2011; 24: 12-17.