Idiopathic Intracranial Hypertension (Pseudotumor Cerebri): Recognition, Treatment, and Ongoing Management
HEADACHE (JR COUCH, SECTION EDITOR)
First Online: 08 November 2012 DOI:
Cite this article as: Thurtell, M.J. & Wall, M. Curr Treat Options Neurol (2013) 15: 1. doi:10.1007/s11940-012-0207-4 Opinion statement
Idiopathic intracranial hypertension (IIH, pseudotumor cerebri) is a syndrome of elevated intracranial pressure of unknown cause that occurs predominantly in obese women of childbearing age. It is a diagnosis of exclusion and, therefore, other causes of increased intracranial pressure must be sought with history, imaging, and cerebrospinal fluid examination before the diagnosis can be made. IIH produces symptoms and signs of increased intracranial pressure, including papilledema. If untreated, papilledema can cause progressive irreversible visual loss and optic atrophy. The treatment approach depends on the severity and time course of symptoms and visual loss, as determined by formal visual field testing. The main goals of treatment are alleviation of symptoms, including headache, and preservation of vision. All overweight IIH patients should be encouraged to enter a weight-management program with a goal of 5–10 % weight loss, along with a low-salt diet. When there is mild visual loss, medical treatment with acetazolamide should be initiated. Other medical treatments can be added or substituted when acetazolamide is insufficient as monotherapy or poorly tolerated. When visual loss is more severe or rapidly progressive, surgical interventions, such as optic nerve sheath fenestration or cerebrospinal fluid shunting, may be required to prevent further irreversible visual loss. The choice of intervention depends on the relative severity of symptoms and visual loss, as well as local expertise. At present, the role of transverse venous sinus stenting remains unclear. Although there are no evidence-based data to guide therapy, there is an ongoing randomized double-blind placebo-controlled treatment trial, investigating diet and acetazolamide therapy for IIH.
Keywords Idiopathic intracranial hypertension Treatment Recognition Management Pseudotumor cerebri Papilledema Vision loss Acetazolamide Topiramate Optic nerve sheath fenestration Cerebrospinal fluid shunting References and Recommended Reading Papers of particular interest, published recently, have been highlighted as: • Of importance
Wall M, George D. Idiopathic intracranial hypertension: a prospective study of 50 patients. Brain. 1991;114:155–80.
Giuseffi V, Wall M, Siegel PZ, Rojas PB. Symptoms and disease associations in idiopathic intracranial hypertension (pseudotumor cerebri): a case-control study. Neurology. 1991;41:239–44.
Wall M. The headache profile of idiopathic intracranial hypertension. Cephalagia. 1990;10:331–5.
Friedman DI, Rausch EA. Headache diagnoses in patients with treated idiopathic intracranial hypertension. Neurology. 2002;58:1551–3.
Durcan FJ, Corbett JJ, Wall M. The incidence of pseudotumor cerebri: population studies in Iowa and Louisiana. Arch Neurol. 1988;45:875–7.
Daniels AB, Liu GT, Volpe NJ, et al. Profiles of obesity, weight gain, and quality of life in idiopathic intracranial hypertension (pseudotumor cerebri). Am J Ophthalmol. 2007;143:635–41.
Sinclair AJ, Burdon MA, Nightingale PG, et al. Low energy diet and intracranial pressure in women with idiopathic intracranial hypertension: prospective cohort study. BMJ. 2010;341:c2701. In this prospective cohort study, the Authors found that weight loss with a low-energy diet produced a significant improvement in intracranial pressure, symptoms, and papilledema.
Newborg B. Pseudotumor cerebri treated by rice reduction diet. Arch Intern Med. 1974;133:802–7.
Johnson LN, Krohel GB, Madsen RW, March Jr GA. The role of weight loss and acetazolamide in the treatment of idiopathic intracranial hypertension (pseudotumor cerebri). Ophthalmology. 1998;105:2313–7.
Wong R, Madill SA, Pandey P, Riordan-Eva P. Idiopathic intracranial hypertension: the association between weight loss and the requirement for systemic treatment. BMC Ophthalmol. 2007;7:15.
Ball AK, Howman A, Wheatley K, et al. A randomised controlled trial of treatment for idiopathic intracranial hypertension. J Neurol. 2011;258:874–81.
Idiopathic Intracranial Hypertension Treatment Trial (IIHTT). Available at:
. Accessed September 2012.
Lee AG, Pless M, Falardeau J, et al. The use of acetazolamide in idiopathic intracranial hypertension during pregnancy. Am J Ophthalmol. 2005;139:855–9.
Falardeau J, Lobb BM, Golden S, et al. The use of acetazolamide during pregnancy in intracranial hypertension patients. J Neuroophthalmol. 2012 (in press).
Tawil R, Moxley 3rd RT, Griggs RC. Acetazolamide-induced nephrolithiasis: implications for treatment of neuromuscular disorders. Neurology. 1993;43:1105–6.
Lichter PR. Reducing side effects of carbonic anhydrase inhibitors. Ophthalmology. 1981;88:266–9.
Celebisoy N, Gökçay F, Sirin H, Akyürekli O. Treatment of idiopathic intracranial hypertension: topiramate vs acetazolamide, an open-label study. Acta Neurol Scand. 2007;116:322–7.
Schoeman JF. Childhood pseudotumor cerebri: clinical and intracranial pressure response to acetazolamide and furosemide treatment in a case series. J Child Neurol. 1994;9:130–4.
Liu GT, Kay MD, Bienfang DC, Schatz NJ. Pseudotumor cerebri associated with corticosteroid withdrawal in inflammatory bowel disease. Am J Ophthalmol. 1994;117:352–7.
Neville BG, Wilson J. Benign intracranial hypertension following corticosteroid withdrawal in childhood. BMJ. 1970;3(5722):554–6.
Thambisetty M, Lavin PJ, Newman NJ, Biousse V. Fulminant idiopathic intracranial hypertension. Neurology. 2007;68:229–32.
Liu GT, Glaser JS, Schatz NJ. High-dose methylprednisolone and acetazolamide for visual loss in pseudotumor cerebri. Am J Ophthalmol. 1994;118:88–96.
De Simone R, Marano E, Fiorillo C, et al. Sudden re-opening of collapsed transverse sinuses and longstanding clinical remission after a single lumbar puncture in a case of idiopathic intracranial hypertension: pathogenetic implications. Neurol Sci. 2005;25:342–4.
Sugerman HJ, Felton 3rd WL, Sismanis A, et al. Gastric surgery for pseudotumor cerebri associated with severe obesity. Ann Surg. 1999;229:634–40.
Fridley J, Foroozan R, Sherman V, et al. Bariatric surgery for the treatment of idiopathic intracranial hypertension. J Neurosurg. 2011;114:34–9.
Johnston I, Besser M, Morgan MK. Cerebrospinal fluid diversion in the treatment of benign intracranial hypertension. J Neurosurg. 1988;69:195–202.
Rosenberg ML, Corbett JJ, Smith C, et al. Cerebrospinal fluid diversion procedures in pseudotumor cerebri. Neurology. 1993;43:1071–2.
Eggenberger ER, Miller NR, Vitale S. Lumboperitoneal shunt for the treatment of pseudotumor cerebri. Neurology. 1996;46:1524–30.
Burgett RA, Purvin VA, Kawasaki A. Lumboperitoneal shunting for pseudotumor cerebri. Neurology. 1997;49:734–9.
Maher CO, Garrity JA, Meyer FB. Refractory idiopathic intracranial hypertension treated with stereotactically planned ventriculoperitoneal shunt placement. Neurosurg Focus. 2001;10:E1.
Bynke G, Zemack G, Bynke H, Romner B. Ventriculoperitoneal shunting for idiopathic intracranial hypertension. Neurology. 2004;63:1314–6.
McGirt MJ, Woodworth G, Thomas G, et al. Cerebrospinal fluid shunt placement for pseudotumor cerebri-associated intractable headache: predictors of treatment response and an analysis of long-term outcomes. J Neurosurg. 2004;101:627–32.
Corbett JJ, Nerad JA, Tse DT, Anderson RL. Results of optic nerve sheath fenestration for pseudotumor cerebri: the lateral orbitotomy approach. Arch Ophthalmol. 1988;106:1391–7.
Sergott RC, Savino PJ, Bosley TM. Modified optic nerve sheath decompression provides long-term visual improvement for pseudotumor cerebri. Arch Ophthalmol. 1988;106:1384–90.
Berman EL, Wirtschafter JD. Improvement of optic nerve head appearance after surgery for pseudotumor cerebri. JAMA. 1992;267:1130.
Kelman SE, Heaps R, Wolf A, Elman MJ. Optic nerve decompression surgery improves visual function in patients with pseudotumor cerebri. Neurosurgery. 1992;30:391–5.
Chandrasekaran S, McCluskey P, Minassian D, Assaad N. Visual outcomes for optic nerve sheath fenestration in pseudotumour cerebri and related conditions. Clin Exp Ophthalmol. 2006;34:661–5.
Yazici Z, Yazici B, Tuncel E. Findings of magnetic resonance imaging after optic nerve sheath decompression in patients with idiopathic intracranial hypertension. Am J Ophthalmol. 2007;144:429–35.
Wilkes BN, Siatkowski RM. Progressive optic neuropathy in idiopathic intracranial hypertension after optic nerve sheath fenestration. J Neuroophthalmol. 2009;29:281–3.
Kaye AH, Galbraith JE, King J. Intracranial pressure following optic nerve decompression for benign intracranial hypertension. J Neurosurg. 1981;55:453–6.
Jacobson EE, Johnston IH, McCluskey P. The effect of optic nerve sheath decompression on CSF dynamics in pseudotumour cerebri and related conditions. J Clin Neurosci. 1999;6:375–7.
Brodsky MC, Rettele GA. Protracted postsurgical blindness with visual recovery following optic nerve sheath fenestration. Arch Ophthalmol. 1998;116:107–9.
Flynn WJ, Westfall CT, Weisman JS. Transient blindness after optic nerve sheath fenestration. Am J Ophthalmol. 1994;117:678–9.
Mauriello Jr JA, Shaderowfsky P, Gizzi M, Frohman L. Management of visual loss after optic nerve sheath decompression in patients with pseudotumor cerebri. Ophthalmology. 1995;102:441–5.
Farb RI, Vanek I, Scott JN, et al. Idiopathic intracranial hypertension: the prevalence and morphology of sinovenous stenosis. Neurology. 2003;60:1418–24.
King JO, Mitchell PJ, Thomson KR, Tress BM. Cerebral venography and manometry in idiopathic intracranial hypertension. Neurology. 1995;45:2224–8.
King JO, Mitchell PJ, Thomson KR, Tress BM. Manometry combined with cervical puncture in idiopathic intracranial hypertension. Neurology. 2002;58:26–30.
Higgins JN, Pickard JD. Lateral sinus stenosis in idiopathic intracranial hypertension resolving after CSF diversion. Neurology. 2004;62:1907–8.
Bateman GA, Stevens SA, Stimpson J. A mathematical model of idiopathic intracranial hypertension incorporating increased arterial inflow and variable venous outflow collapsibility. J Neurosurg. 2009;110:446–56.
Owler BK, Parker G, Halmagyi GM, et al. Pseudotumor cerebri syndrome: venous sinus obstruction and its treatment with stent placement. J Neurosurg. 2003;98:1045–55.
Higgins JN, Cousins C, Owler BK, et al. Idiopathic intracranial hypertension: 12 cases treated by venous sinus stenting. J Neurol Neurosurg Psychiatry. 2003;74:1662–6.
Owler BK, Parker G, Halmagyi GM, et al. Cranial venous outflow obstruction and pseudotumor cerebri syndrome. Adv Tech Stand Neurosurg. 2005;30:107–74.
Donnet A, Metellus P, Levrier O, et al. Endovascular treatment of idiopathic intracranial hypertension: clinical and radiologic outcome of 10 consecutive patients. Neurology. 2008;70:641–7.
Bussière M, Falero R, Nicolle D, et al. Unilateral transverse sinus stenting of patients with idiopathic intracranial hypertension. AJNR. 2010;31:645–50.
Ahmed RM, Wilkinson M, Parker GD, et al. Transverse sinus stenting for idiopathic intracranial hypertension: a review of 52 patients and of model predictions. AJNR. 2011;32:1408–14. In this large retrospective case series, the Authors report their experience in treating IIH with cerebral venous sinus stenting. The study findings suggest that stenting may have a role in the treatment of selected IIH patients, but that significant complications can occur.
Avery RA, Shah SS, Licht DJ, et al. Reference range for cerebrospinal fluid opening pressure in children. N Engl J Med. 2010;363:891–3.
Lee MW, Vedanarayanan VV. Cerebrospinal fluid opening pressure in children: experience in a controlled setting. Pediatr Neurol. 2011;45:238–40. In this retrospective study, the Authors report the range of CSF opening pressures obtained from lumbar puncture of pediatric patients in a controlled, uniform setting. They conclude that while the reference range for opening pressure was closer to that for adults than previously appreciated, values above 20 cmH
O should not necessarily be considered abnormal.
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