Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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June 1, 2015
Idiopathic Intracranial Hypertension
- Background: Idiopathic intracranial hypertension is a clinical syndrome of raised intracranial pressure without any identifiable cause. It typically affects obese women of child-bearing age.
- Clinical Features: Headaches, transient visual disturbances, pulse-synchronous tinnitus. Clinical diagnosis is supported by high opening pressures on lumbar puncture. If untreated, may result in visual loss.
- Key Diagnostic Features: Normal/decreased size of the ventricular system, partially empty sella, flattening/indentation of the posterior sclera, prominent optic nerve sheaths, tortuosity of the optic nerves. Bilateral transverse sinus stenosis is associated with 90% of cases; however, whether this is a cause or effect relationship is debated.
- DDx:
- Mild cerebral edema
- Empty sella syndrome
- Hypoplastic dural venous sinuses
- Treatment: Treatment goals are restoration of visual acuity and resolution of the papilledema. Treatment strategies include:
- Weight loss
- Acetazolamide (furosemide can be added)
- Therapeutic lumbar punctures - lumboperitoneal shunt
- Optic nerve sheath fenestration
- Venous stenting has been also proposed if there is no improvement or if there is rapid worsening of visual acuity, but it is still not widely used.