Treatment of Pseudotumor Cerebri (Idiopathic Intracranial Hypertension)

Initial Assessment and Management

The American College of Radiology recommends MRI of the head and orbits as the most useful imaging modality for initial evaluation of patients presenting with papilledema and signs of raised intracranial pressure 1, 2
CT venography (CTV) or MR venography (MRV) should be performed to evaluate cerebral venous sinuses 2
Diagnostic criteria for pseudotumor cerebri include papilledema, normal neurological examination, normal brain parenchyma on imaging, normal cerebrospinal fluid composition, and elevated lumbar puncture opening pressure 2, 3

The American Academy of Neurology recommends weight loss as the first-line treatment for pseudotumor cerebri in overweight patients, while acetazolamide should be initiated as the primary medical therapy for patients with mild visual loss 4
Acetazolamide is the first-line medication for patients with mild visual loss, with a starting dose that can be gradually increased as needed and tolerated 4
Ventriculoperitoneal (VP) shunt should be the preferred CSF diversion procedure due to lower reported revision rates 4, 5
Optic Nerve Sheath Fenestration (ONSF) is an effective and safe procedure that may be repeated if initially unsuccessful, with fewer complications than CSF diversion procedures 5
Neurovascular stenting may lead to improvement in symptoms but has potential complications including headache, stent-adjacent stenosis, and rare serious complications 4, 5

Medications that might cause or exacerbate IIH, such as tetracyclines, vitamin A, retinoids, steroids, growth hormone, thyroxine, and lithium, should be identified and discontinued if possible 2
Serial lumbar punctures are not recommended for management of IIH 5

Treatment failure rates include worsening vision after stabilization in 34% of patients at 1 year and 45% at 3 years 4
Failure to improve headache occurs in one-third to one-half of treated patients 4

ONSF should be performed only by an experienced clinician trained in this technique 5
Patients should inform the relevant driving agency following VP shunt placement, as recommended by the relevant medical society 4

Topiramate may help with weight loss by suppressing appetite and have an effect on reducing intracranial pressure through carbonic anhydrase inhibition, and Zonisamide may be an alternative where topiramate has excessive side effects 6
CSF shunting to exclusively treat headache has limited evidence, with 68% of patients continuing to have headaches at 6 months and 79% by 2 years 6
Patients with IIH often develop migrainous headaches superimposed on headaches from raised intracranial pressure, requiring specific treatment approaches 6
Once papilloedema has resolved, visual monitoring within hospital services may no longer be required, but caution is needed for asymptomatic patients at presentation who may remain asymptomatic during recurrence 6

The American Academy of Neurosurgery recommends avoiding the use of acetazolamide combined with furosemide in preterm infants, as this combination increases mortality and neurological morbidity 7
The American Academy of Neurosurgery also advises against performing ventricular puncture routinely, as it increases the risk of CSF infection and loculated hydrocephalus 7
The National Blood Service guidelines state that patients should not donate blood during or for at least 1 year following discontinuation of acetazolamide therapy if they were on retinoid-class medications, as recommended by the British Association of Dermatologists 8