Idiopathic intracranial hypertension (Pseudotumor cerebri)

Abstract

Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a disorder of elevated intracranial pressure of unknown cause. Patients present with daily headache, pulse-synchronous tinnitus, transient visual obscurations, papilledema with its associated visual loss, and diplopia from sixth nerve paresis. Many disease associations have been alleged, but few besides obesity, hypervitaminosis A and related compounds, steroid withdrawal, and female gender have been proven. Although absorption of cerebrospinal fluid (CSF) occurs through arachnoid granulations and extracranial lymphatics, outflow resistance is increased in IIH; therefore, intracranial pressure must increase for CSF to be absorbed. The mainstays of medical treatment are a reduced-sodium weight-reduction program and acetazolamide. If patients fail medical therapy, surgical procedures, most commonly optic nerve sheath fenestration and CSF shunting, are employed. The main morbidity of IIH is visual loss. This is present in most patients and can usually be reversed if recognized early in the course of the disease and treated.

Headache in patients with idiopathic intracranial hypertension: a pilot study to assess applicability of ICHD-2 diagnostic criteria

“…Secondary intracranial hypertension occurs in a large variety of disorders, such as intracranial mass lesions (tumor, abscess), dural venous sinus thrombosis, traumatic brain injury, ischemic or hemorrhagic stroke, dural arteriovenous fistula, hydrocephalus [3]. An intracranial pressure at lumbar puncture in the recumbent position higher than 200 mmH 2 O in adults is usually accepted as a marker of IIH diagnosis.…”

Idiopathic Intracranial Hypertension in Pregnancy Treated with Serial Lumbar Punctures

“…Other medical treatment options besides acetazolamide that may be indicated for IIH include furosemide, topiramate, and corticosteroids [6,9,11]. Furosemide reduces intracranial pressure by both diuresis and reducing the transport of sodium ions in the brain.…”

“…Topiramate may also be efficacious and well tolerated in the treatment of IIH because of its carbonic anhydrase activity and weight reducing effect [8,9,11,12]. However, side effects include nephrolithiasis and fetal deformities [13].…”

Transient, unilateral, complete, oculomotor palsy in an adult patient with idiopathic intracranial hypertension

“…Typical presentation consists of daily headache, pulse-synchronous tinnitus, transient blurred vision, papilledema with associated visual loss, and diplopia from abducens nerve paresis. (1) Several conditions have been suspected, but only a few, including obesity and weight gain during the 12 months before diagnosis, steroid withdrawal, female gender, hypervitaminosis A and related compounds, have been proven. (2)(3)(4) The treatment is focused on the correction of the presumed cause, including a low-sodium diet, diuretics and a weight-reduction program.…”