The term idiopathic intracranial hypertension (IIH) has been used interchangeably in the literature with pseudotumor cerebri. Pseudotumor cerebri syndrome (PTCS) is now used to denote idiopathic and secondary etiologies of increased intracranial hypertension. Common symptoms of elevated intracranial pressure (ICP) are headache, transient visual obscurations, and pulsatile tinnitus. Visual acuity may range from normal to no light perception. The visual acuity is not related to the degree of papilledema, except for atrophic papilledema in which the vision would be invariably poor. Concentric enlargement of the blind spot is the most common defect, followed by isopter constriction and loss of the inferior nasal quadrant of the visual field with a nasal step. Color defects usually involve red-green abnormalities. No afferent pupillary defect is detected in most instances of bilateral papilledema. One-third of patients have horizontal diplopia. Papilledema is clinically defined as optic disc swelling resulting only from increased ICP, which is equal to or greater than 250 mmH2O in adults while in a lateral recumbent position. Optical coherence tomography (OCT) can be used as an adjunct to ophthalmoscopy to monitor the severity and evolution of papilledema.

The two major goals of therapy in PTCS, a term that includes idiopathic (IIH) and secondary etiologies of increased intracranial hypertension, are to prevent visual loss and treat and prevent headaches. The mainstay of medical treatment of PTCS is weight loss. The Idiopathic Intracranial Hypertension Treatment Trial is currently an ongoing prospective randomized, double-blinded, placebo-controlled trial to compare the efficacy of acetazolamide (up to 4 g/day) added to a low-sodium, weight reduction diet versus dieting alone or preventing or restoring visual loss. If headaches develop, then antimigraine medications may be added.

Indications for surgery include the following: (1) progressive visual loss despite maximal medical treatment, (2) severe or sudden visual loss at onset with an afferent pupillary defect or signs of advancing optic nerve dysfunction, and (3) severe papilledema causing macular edema or exudates. Optic nerve sheath decompression (ONSD) has been shown to be safe and effective in treating vision in PTCS. ONSD may not be the treatment of choice for those who have progressive visual loss and intractable headaches, which are better managed by ventriculoperitoneal (VP) or lumboperitoneal (LP) shunting. Because of the high rate of complications and failures following VP or LP shunting, bariatric surgery may be an effective alternative in severely obese patients with PTCS. Because of increasing evidence of nonthrombotic dural venous sinus stenosis, endovascular stenting may be an option for treatment of PTCS.

The management of PTCS in pregnancy is similar to that in nonpregnant adults. In children, the criteria for elevated CSF opening pressure are greater than 280 mmH2O. The management of PTCS otherwise is similar to that in adults.


Retinal Nerve Fiber Layer Visual Field Defect Retinal Nerve Fiber Layer Thickness Venous Sinus Idiopathic Intracranial Hypertension 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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© Springer Science+Business Media New York 2014

Authors and Affiliations

  1. 1.Department of Neurology, Neuro-OphthalmologyUniversity of Nevada School of MedicineRenoUSA

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