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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.

PATHOGENESIS
Edwards L, Constantinescu C. Cytokines in idiopathic intracranial hypertension
CSF
. Headache. 2010 Feb;50(2):323-5. doi: 10.1111/j.1526-4610.2009.01592.x. Epub
2009 Dec 21. PubMed PMID: 20039956.

Dhungana S, Sharrack B, Woodroofe N. Cytokines and chemokines in idiopathic
intracranial hypertension
. Headache. 2009 Feb;49(2):282-5. doi:
10.1111/j.1526-4610.2008.001329.x. PubMed PMID: 19222599.

Borsody M, Semenov I, Carroll K, Kessler A, Dubow J, Olson E, Stern J, Barion
A, Hammond C, Van Stavern G, Raizer J, White R, Leenen F. The relation of brain
ouabain-like compounds and idiopathic intracranial hypertension
. Headache. 2006 Sep;46(8):1255-60. PubMed PMID: 16942469.

RISK FACTORS
Hannerz J, Ericson K. The relationship between idiopathic intracranial
hypertension and obesity
. Headache. 2009 Feb;49(2):178-84. doi:
10.1111/j.1526-4610.2008.01240.x. PubMed PMID: 19222591.

UNIQUE OR SPECIAL SCENARIOS
Evans RW, Lee AG. Idiopathic intracranial hypertension in pregnancy. Headache.
2010 Oct;50(9):1513-5. doi: 10.1111/j.1526-4610.2010.01760.x. PubMed PMID:
20958297.

Halsey JH. Pseudotumor cerebri without papilledema. Headache. 2009
Jul;49(7):1073-4. doi: 10.1111/j.1526-4610.2009.01468.x. Epub 2009 May 27. PubMed
PMID: 19486174.

Digre KB, Nakamoto BK, Warner JE, Langeberg WJ, Baggaley SK, Katz BJ. A
comparison of idiopathic intracranial hypertension with and without papilledema.
Headache. 2009 Feb;49(2):185-93. doi: 10.1111/j.1526-4610.2008.01324.x. PubMed PMID: 19222592.

Jacome DE. Idiopathic intracranial hypertension and hemophilia A. Headache.
2001 Jun;41(6):595-8. PubMed PMID: 11437898.

Evans RW, Friedman DI. Expert opinion: the management of pseudotumor cerebri
during pregnancy
. Headache. 2000 Jun;40(6):495-7. PubMed PMID: 10849049.

UNUSUAL MANIFESTATIONS
Capobianco DJ, Brazis PW, Cheshire WP. Idiopathic intracranial hypertension
and seventh nerve palsy
. Headache. 1997 May;37(5):286-8. Review. PubMed PMID:
9195767.

Winner P, Bello L. Idiopathic intracranial hypertension in a young child
without visual symptoms or signs
. Headache. 1996 Oct;36(9):574-6. PubMed PMID:
8916568.

Chari C, Rao NS. Benign intracranial hypertension--its unusual
manifestations
. Headache. 1991 Oct;31(9):599-600. PubMed PMID: 1774176.

Landan I, Policherla H, McLaurin J. Complete external ophthalmoplegia in a
case of pseudotumor cerebri
. Headache. 1987 Nov;27(10):573-4. PubMed PMID:
3692820.

EVALUATION AND DIAGNOSIS
O'Connor W, Silberstein SD. MRI of a brain under pressure. Headache. 2005
Jan;45(1):68-9. PubMed PMID: 15663615.

Evans RW, Silberstein SD. Diagnostic testing for chronic daily headache.
Headache. 2002 Jun;42(6):556-9. PubMed PMID: 12167151.

Lewis DW, Dorbad D. The utility of neuroimaging in the evaluation of children
with migraine or chronic daily headache who have normal neurological
examinations
. Headache. 2000 Sep;40(8):629-32. PubMed PMID: 10971658.

Kan L, Nagelberg J, Maytal J. Headaches in a pediatric emergency department:
etiology, imaging, and treatment.
Headache. 2000 Jan;40(1):25-9. PubMed PMID:
10759899.

DIFFERENTIAL DIAGNOSIS
Knash ME, Goadsby PJ. Migraine misdiagnosed as idiopathic intracranial
hypertension
. Headache. 2011 Nov-Dec;51(10):1537-9. doi:
10.1111/j.1526-4610.2011.01971.x. Epub 2011 Aug 29. PubMed PMID: 21883196.

Ertekin V, Selimo─člu MA, Tan H. Pseudotumor cerebri due to hypervitaminosis A
or hypervitaminosis D or both in Alagille syndrome
. Headache. 2010
Jan;50(1):152-3. doi: 10.1111/j.1526-4610.2009.01489.x. Epub 2009 Jul 8. PubMed
PMID: 19619235.

Thapa R, Mallick D, Biswas B. Pseudotumor cerebri in childhood hepatitis E
virus infection
. Headache. 2009 Apr;49(4):610-1. doi:
10.1111/j.1526-4610.2009.01367.x. Epub 2009 Feb 25. PubMed PMID: 19245388.

Lardizabal DV. Intracranial hypertension and levofloxacin: a case report.
Headache. 2009 Feb;49(2):300-1. doi: 10.1111/j.1526-4610.2008.01212.x. Epub 2008
Jul 21. PubMed PMID: 18647180.

Nawashiro H. Idiopathic intracranial hypertension associated with
sulphasalazine treatment
. Headache. 2008 Oct;48(9):1383-4. doi:
10.1111/j.1526-4610.2008.01213.x. Epub 2008 Jul 21. PubMed PMID: 18647179.

Rourke T, Davies S, Samandouras G. Serial LPS without serial imaging in
idiopathic intracranial hypertension: a lesson learned
. Headache. 2008
Apr;48(4):630-2. doi: 10.1111/j.1526-4610.2008.01058.x. Epub 2008 Feb 20. PubMed
PMID: 18298581.

Sevgi E, Yalcin G, Kansu T, Varli K. Drug induced intracranial hypertension
associated with sulphasalazine treatment
. Headache. 2008 Feb;48(2):296-8. Epub
2007 Dec 7. PubMed PMID: 18070060.

Jacob S, Rajabally YA. Intracranial hypertension induced by rofecoxib.
Headache. 2005 Jan;45(1):75-6. PubMed PMID: 15663617.

Herwig U, Sturzenegger M. Hyperthyroidism mimicking increased intracranial
pressure
. Headache. 1999 Mar;39(3):228-30. PubMed PMID: 15613219.

Adelman JU. Headaches and papilledema secondary to dural arteriovenous
malformation
. Headache. 1998 Sep;38(8):621-3. PubMed PMID: 11398307.

Evans RW, Dulli D. Pseudo-pseudotumor cerebri. Headache. 2001
Apr;41(4):416-8. PubMed PMID: 11318891.

Swerdlow RH, Hanna GR. Behcet's disease: presentation with sagittal sinus
thrombosis diagnosed noninvasively
. Headache. 1996 Feb;36(2):115-8. PubMed PMID: 8742686.

Uyama E, Ueno N, Uchino M, Narahara T, Owada M, Taketomi T, Ando M. Headache
associated with aseptic meningeal reaction as clinical onset of Fabry's disease
. Headache. 1995 Sep;35(8):498-501. PubMed PMID: 7591747.

Hannerz J, Greitz D, Ericson K. Periorbital venous vasculitis, intracranial
hypertension and empty sella
. Headache. 1994 Feb;34(2):95-8. PubMed PMID:
8163374.

Silberstein SD, Marcelis J. Headache associated with changes in intracranial
pressure
. Headache. 1992 Feb;32(2):84-94. Review. PubMed PMID: 1551793.

Askmark H, Lundberg PO, Olsson S. Drug-related headache. Headache. 1989
Jul;29(7):441-4. PubMed PMID: 2759851.

Alvarez-Cermeño JC, Fernández JM, O'Neill A, Moral L, Saiz-Ruiz J.
Lithium-induced headache. Headache. 1989 Apr;29(4):246-7. PubMed PMID: 2497083.

COMPLICATIONS
Patel BA, Williams NR, Pritchard PB 3rd. Unique Case of "Post-Lumbar Puncture
Headache"
. Headache. 2013 Jan 8. doi: 10.1111/head.12005. [Epub ahead of print]
PubMed PMID: 23298181.

Graber JJ, Racela R, Henry K. Cerebellar tonsillar herniation after weight
loss in a patient with idiopathic intracranial hypertension
. Headache. 2010
Jan;50(1):146-8. doi: 10.1111/j.1526-4610.2009.01546.x. Epub 2009 Oct 8. PubMed
PMID: 19817881.

Loh Y, Labutta RJ, Urban ES. Idiopathic intracranial hypertension and
postlumbar puncture headache
. Headache. 2004 Feb;44(2):170-3. PubMed PMID:
14756857.

TREATMENT
Marmura MJ, Hopkins M, Andrel J, Young WB, Biondi DM, Rupnow MF, Armstrong RB.
Electronic medical records as a research tool: evaluating topiramate use at a
headache center
. Headache. 2010 May;50(5):769-78. doi:
10.1111/j.1526-4610.2010.01624.x. Epub 2010 Mar 4. PubMed PMID: 20236335.

Palacio E, Rodero L, Pascual J. Topiramate-responsive headache due to
idiopathic intracranial hypertension in Behçet syndrome
. Headache. 2004
May;44(5):436-7. PubMed PMID: 15147251.

Pagan FL, Restrepo L, Balish M, Patwa HS, Houff S. A new drug for an old
condition?
Headache. 2002 Jul-Aug;42(7):695-6. PubMed PMID: 12482228.

TREATMENT MODALITIES REQUIRING FURTHER STUDY
Evans RW, Ramadan NM. Are cannabis-based chemicals helpful in headache?
Headache. 2004 Jul-Aug;44(7):726-7. PubMed PMID: 15209699.

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