Pseudotumor Cerebri

Abstract

Pseudotumor cerebri is also known as benign/idiopathic intracranial hypertension. Patients with pseudotumor cerebri are usually young, obese females who may have a history of medication use including tetracycline, steroids, or vitamin A and its derivatives. Symptoms and signs include generalized headache, transient visual obscurations, papilledema, and cranial nerve VI palsy (a nonlocalizing sign).

The examination may also reveal an enlarged blind spot, loss of the inferonasal visual field, and generalized constriction of the visual field in advanced cases.

Appropriate investigations include brain imaging with CT or MRI which may reveal slit-like ventricles. A lumbar puncture is essential, and this usually reveals an elevated opening pressure above 250 mmH₂O and possibly a low protein concentration. In pregnant or postpartum patients, the differential includes venous sinus thrombosis, which must be ruled out with an MRV.

Management includes cessation of the offending agent and treatment with acetazolamide or furosemide. However, the mainstay of long-term management is sustained weight loss. Refractory patients may need a ventriculoperitoneal shunt or an optic nerve sheath fenestration procedure.

There is significant risk of permanent loss of vision in untreated patients; therefore, pseudotumor cerebri is practically a medical emergency and should be appropriately considered in the differential diagnosis of headache.