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Idiopathic Intracranial Hypertension (IIH)

Ivan Garza, MD, FAHS

Dr. Ivan Garza is an Assistant Professor of Neurology at the Mayo Clinic College of Medicine and a Consultant at the Mayo Clinic’s Department of Neurology in Rochester, MN. He is board certified in Neurology by the American Board of Psychiatry and Neurology (ABPN) and holds subspecialty certification in Headache Medicine by the United Council of Neurologic Subspecialties (UCNS).

Idiopathic Intracranial Hypertension (IIH), previously referred to as “pseudotumor cerebri”, is a disorder of increased intracranial pressure of poorly understood pathogenesis. It usually affects young obese women and manifests as headaches and papilledema, the latter of which is the most concerning complication. Other symptoms include transient visual obscurations, pulsatile tinnitus, photopsias, diplopia, and vision loss. The hallmark sign in IIH is papilledema (present in >90%) and a less commonly encountered sign is a unilateral or bilateral abducens paresis which can be seen in approximately 15% of cases. Importantly, because the symptoms and signs of IIH are not specific, multiple other causes of intracranial hypertension need to be ruled out before a diagnosis of IIH can be made. The diagnostic criteria for IIH have evolved from the “Modified Dandy Criteria” and to satisfy these, multiple evaluations are necessary. Brain imaging is mandatory before lumbar puncture, and MRI is the procedure of choice rather than CT. Lumbar puncture is mandatory to confirm diagnosis and help exclude other disorders. An eye exam performed by an ophthalmologist or neuro-ophthalmologist is necessary to exclude causes of disc edema other than intracranial hypertension and should include perimetry, the most useful test to evaluate visual function in IIH.

The primary aims of management are to decrease intracranial pressure and reduce the risk of vision loss, while the secondary goal is to reduce other symptoms such as headache. Weight loss should occur via low calorie diet and regular exercise although bariatric surgery can be considered in IIH with severe obesity. Acetazolamide is the 1st line pharmacologic option and is usually prescribed simultaneously to weight loss. The mechanism through which acetazolamide benefits IIH is likely multifactorial and secondary to a decrease in CSF production via inhibition of carbonic anhydrase and anorexia with weight loss. Second line medications include furosemide, triamterene, and spironolactone among others. Whether topiramate may be similar to acetazolamide in IIH management has not been confirmed in controlled trials. Serial lumbar punctures are not usually recommended but may have a role as temporizing measure in preparation for surgery or in pregnant patients who wish to avoid medical therapy. Surgical indications for the treatment of IIH are progressive visual loss despite maximal tolerated medical therapy or severe visual loss at presentation. The main modalities are optic nerve sheath fenestration, CSF shunting, and venous sinus stenting. Venous sinus stenting, however, can be associated with serious complications and is therefore not recommended routinely. Other surgical options should be considered in preference to venous sinus stenting.

Manuscripts published in Headache pertaining to the syndrome of IIH are outlined below.

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Hannerz J, Ericson K. The relationship between idiopathic intracranial
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Evans RW, Lee AG. Idiopathic intracranial hypertension in pregnancy. Headache.
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Digre KB, Nakamoto BK, Warner JE, Langeberg WJ, Baggaley SK, Katz BJ. A
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Nawashiro H. Idiopathic intracranial hypertension associated with
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Graber JJ, Racela R, Henry K. Cerebellar tonsillar herniation after weight
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Marmura MJ, Hopkins M, Andrel J, Young WB, Biondi DM, Rupnow MF, Armstrong RB.
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Pagan FL, Restrepo L, Balish M, Patwa HS, Houff S. A new drug for an old
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Evans RW, Ramadan NM. Are cannabis-based chemicals helpful in headache?
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