Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate®

Idiopathic intracranial hypertension (pseudotumor cerebri): Prognosis and treatment

Andrew G Lee, MD
Michael Wall, MD
Section Editor
Paul W Brazis, MD
Deputy Editor
Janet L Wilterdink, MD


Idiopathic intracranial hypertension (IIH) is also called pseudotumor cerebri. It is a disorder defined by clinical criteria that include symptoms and signs isolated to those produced by increased intracranial pressure (eg, headache, papilledema, vision loss), elevated intracranial pressure with normal cerebrospinal fluid composition, and no other cause of intracranial hypertension evident on neuroimaging or other evaluations [1].

While once called benign intracranial hypertension, to distinguish it from secondary intracranial hypertension produced by a neoplastic malignancy, it is not a benign disorder. Many patients suffer from intractable, disabling headaches, and there is a risk of severe, permanent vision loss.

Recommendations for the treatment of IIH are limited by a limited number of randomized controlled trials [2,3]. Moreover, the natural history of untreated IIH is uncertain

This topic will discuss the prognosis and treatment of IIH. The epidemiology, pathogenesis, clinical features and diagnosis of this disorder are discussed separately. (See "Idiopathic intracranial hypertension (pseudotumor cerebri): Epidemiology and pathogenesis" and "Idiopathic intracranial hypertension (pseudotumor cerebri): Clinical features and diagnosis".)


No large prospective series describe the natural history of idiopathic intracranial hypertension (IIH). A protracted course lasting months to years appears to be common [4-8]. In most patients, symptoms worsen slowly. With treatment, there is usually gradual improvement and/or stabilization, but not necessarily recovery; many patients have persistent papilledema, elevated intracranial pressure as documented on lumbar puncture, and residual visual field deficits.


Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: May 2017. | This topic last updated: Jun 17, 2015.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  1. Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension. Neurology 2002; 59:1492.
  2. Lueck C, McIlwaine G. Interventions for idiopathic intracranial hypertension. Cochrane Database Syst Rev 2005; :CD003434.
  3. Biousse V, Bruce BB, Newman NJ. Update on the pathophysiology and management of idiopathic intracranial hypertension. J Neurol Neurosurg Psychiatry 2012; 83:488.
  4. Wall M, George D. Idiopathic intracranial hypertension. A prospective study of 50 patients. Brain 1991; 114 ( Pt 1A):155.
  5. Celebisoy N, Seçil Y, Akyürekli O. Pseudotumor cerebri: etiological factors, presenting features and prognosis in the western part of Turkey. Acta Neurol Scand 2002; 106:367.
  6. Wall M. Sensory visual testing in idiopathic intracranial hypertension: measures sensitive to change. Neurology 1990; 40:1859.
  7. Acheson JF. Idiopathic intracranial hypertension and visual function. Br Med Bull 2006; 79-80:233.
  8. Salman MS, Kirkham FJ, MacGregor DL. Idiopathic "benign" intracranial hypertension: case series and review. J Child Neurol 2001; 16:465.
  9. Thambisetty M, Lavin PJ, Newman NJ, Biousse V. Fulminant idiopathic intracranial hypertension. Neurology 2007; 68:229.
  10. Liu GT, Glaser JS, Schatz NJ. High-dose methylprednisolone and acetazolamide for visual loss in pseudotumor cerebri. Am J Ophthalmol 1994; 118:88.
  11. Kidron D, Pomeranz S. Malignant pseudotumor cerebri. Report of two cases. J Neurosurg 1989; 71:443.
  12. Corbett JJ, Savino PJ, Thompson HS, et al. Visual loss in pseudotumor cerebri. Follow-up of 57 patients from five to 41 years and a profile of 14 patients with permanent severe visual loss. Arch Neurol 1982; 39:461.
  13. Rowe FJ, Sarkies NJ. Visual outcome in a prospective study of idiopathic intracranial hypertension. Arch Ophthalmol 1999; 117:1571.
  14. Radhakrishnan K, Ahlskog JE, Cross SA, et al. Idiopathic intracranial hypertension (pseudotumor cerebri). Descriptive epidemiology in Rochester, Minn, 1976 to 1990. Arch Neurol 1993; 50:78.
  15. Bruce BB, Preechawat P, Newman NJ, et al. Racial differences in idiopathic intracranial hypertension. Neurology 2008; 70:861.
  16. NORDIC Idiopathic Intracranial Hypertension Study Group Writing Committee, Wall M, McDermott MP, et al. Effect of acetazolamide on visual function in patients with idiopathic intracranial hypertension and mild visual loss: the idiopathic intracranial hypertension treatment trial. JAMA 2014; 311:1641.
  17. Stiebel-Kalish H, Kalish Y, Lusky M, et al. Puberty as a risk factor for less favorable visual outcome in idiopathic intracranial hypertension. Am J Ophthalmol 2006; 142:279.
  18. Bruce BB, Kedar S, Van Stavern GP, et al. Idiopathic intracranial hypertension in men. Neurology 2009; 72:304.
  19. Bruce BB, Kedar S, Van Stavern GP, et al. Atypical idiopathic intracranial hypertension: normal BMI and older patients. Neurology 2010; 74:1827.
  20. Saindane AM, Bruce BB, Riggeal BD, et al. Association of MRI findings and visual outcome in idiopathic intracranial hypertension. AJR Am J Roentgenol 2013; 201:412.
  21. Taktakishvili O, Shah VA, Shahbaz R, Lee AG. Recurrent idiopathic intracranial hypertension. Ophthalmology 2008; 115:221.
  22. Shah VA, Kardon RH, Lee AG, et al. Long-term follow-up of idiopathic intracranial hypertension: the Iowa experience. Neurology 2008; 70:634.
  23. Kesler A, Hadayer A, Goldhammer Y, et al. Idiopathic intracranial hypertension: risk of recurrences. Neurology 2004; 63:1737.
  24. Ko MW, Chang SC, Ridha MA, et al. Weight gain and recurrence in idiopathic intracranial hypertension: a case-control study. Neurology 2011; 76:1564.
  25. 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.
  26. Newborg B. Pseudotumor cerebri treated by rice reduction diet. Arch Intern Med 1974; 133:802.
  27. Kupersmith MJ, Gamell L, Turbin R, et al. Effects of weight loss on the course of idiopathic intracranial hypertension in women. Neurology 1998; 50:1094.
  28. Johnson LN, Krohel GB, Madsen RW, March GA Jr. The role of weight loss and acetazolamide in the treatment of idiopathic intracranial hypertension (pseudotumor cerebri). Ophthalmology 1998; 105:2313.
  29. Glueck CJ, Golnik KC, Aregawi D, et al. Changes in weight, papilledema, headache, visual field, and life status in response to diet and metformin in women with idiopathic intracranial hypertension with and without concurrent polycystic ovary syndrome or hyperinsulinemia. Transl Res 2006; 148:215.
  30. Sugerman HJ, Felton WL 3rd, Salvant JB Jr, et al. Effects of surgically induced weight loss on idiopathic intracranial hypertension in morbid obesity. Neurology 1995; 45:1655.
  31. Sugerman HJ, Felton WL 3rd, Sismanis A, et al. Gastric surgery for pseudotumor cerebri associated with severe obesity. Ann Surg 1999; 229:634.
  32. Chandra V, Dutta S, Albanese CT, et al. Clinical resolution of severely symptomatic pseudotumor cerebri after gastric bypass in an adolescent. Surg Obes Relat Dis 2007; 3:198.
  33. Nadkarni T, Rekate HL, Wallace D. Resolution of pseudotumor cerebri after bariatric surgery for related obesity. Case report. J Neurosurg 2004; 101:878.
  34. McCarthy KD, Reed DJ. The effect of acetazolamide and furosemide on cerebrospinal fluid production and choroid plexus carbonic anhydrase activity. J Pharmacol Exp Ther 1974; 189:194.
  35. Gücer G, Viernstein L. Long-term intracranial pressure recording in the management of pseudotumor cerebri. J Neurosurg 1978; 49:256.
  36. Matthews YY. Drugs used in childhood idiopathic or benign intracranial hypertension. Arch Dis Child Educ Pract Ed 2008; 93:19.
  37. Youroukos S, Psychou F, Fryssiras S, et al. Idiopathic intracranial hypertension in children. J Child Neurol 2000; 15:453.
  38. Warman R. Management of pseudotumor cerebri in children. Int Pediatr 2000; 15:147.
  39. Lee AG, Anderson R, Kardon RH, Wall M. Presumed "sulfa allergy" in patients with intracranial hypertension treated with acetazolamide or furosemide: cross-reactivity, myth or reality? Am J Ophthalmol 2004; 138:114.
  40. Platt D, Griggs RC. Use of acetazolamide in sulfonamide-allergic patients with neurologic channelopathies. Arch Neurol 2012; 69:527.
  41. Lee AG, Pless M, Falardeau J, et al. The use of acetazolamide in idiopathic intracranial hypertension during pregnancy. Am J Ophthalmol 2005; 139:855.
  42. Digre KB, Corbett JJ. Pseudotumor cerebri in men. Arch Neurol 1988; 45:866.
  43. Huna-Baron R, Kupersmith MJ. Idiopathic intracranial hypertension in pregnancy. J Neurol 2002; 249:1078.
  44. Digre KB, Varner MW, Corbett JJ. Pseudotumor cerebri and pregnancy. Neurology 1984; 34:721.
  45. 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.
  46. Pagan FL, Restrepo L, Balish M, et al. A new drug for an old condition? Headache 2002; 42:695.
  47. Shah VA, Fung S, Shahbaz R, et al. Idiopathic intracranial hypertension. Ophthalmology 2007; 114:617.
  48. Friedman, DI, Eller, PF. Topiramate for the treatment of idiopathic intracranial hypertension. Headache 2003; 43:592.
  49. Finsterer J, Földy D, Fertl E. Topiramate resolves headache from pseudotumor cerebri. J Pain Symptom Manage 2006; 32:401.
  50. 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.
  51. Corbett JJ, Thompson HS. The rational management of idiopathic intracranial hypertension. Arch Neurol 1989; 46:1049.
  52. Friedman DI, Jacobson DM. Idiopathic intracranial hypertension. J Neuroophthalmol 2004; 24:138.
  53. Rasmussen M. Treatment of elevated intracranial pressure with indomethacin: friend or foe? Acta Anaesthesiol Scand 2005; 49:341.
  54. Förderreuther S, Straube A. Indomethacin reduces CSF pressure in intracranial hypertension. Neurology 2000; 55:1043.
  55. Biousse V, Rucker JC, Vignal C, et al. Anemia and papilledema. Am J Ophthalmol 2003; 135:437.
  56. Friedman DI, Rausch EA. Headache diagnoses in patients with treated idiopathic intracranial hypertension. Neurology 2002; 58:1551.
  57. Wang SJ, Silberstein SD, Patterson S, Young WB. Idiopathic intracranial hypertension without papilledema: a case-control study in a headache center. Neurology 1998; 51:245.
  58. Mathew NT, Ravishankar K, Sanin LC. Coexistence of migraine and idiopathic intracranial hypertension without papilledema. Neurology 1996; 46:1226.
  59. Corbett JJ, Mehta MP. Cerebrospinal fluid pressure in normal obese subjects and patients with pseudotumor cerebri. Neurology 1983; 33:1386.
  60. Evans RW, Friedman DI. Expert opinion: the management of pseudotumor cerebri during pregnancy. Headache 2000; 40:495.
  61. Fonseca PL, Rigamonti D, Miller NR, Subramanian PS. Visual outcomes of surgical intervention for pseudotumour cerebri: optic nerve sheath fenestration versus cerebrospinal fluid diversion. Br J Ophthalmol 2014; 98:1360.
  62. Acheson JF, Green WT, Sanders MD. Optic nerve sheath decompression for the treatment of visual failure in chronic raised intracranial pressure. J Neurol Neurosurg Psychiatry 1994; 57:1426.
  63. Brourman ND, Spoor TC, Ramocki JM. Optic nerve sheath decompression for pseudotumor cerebri. Arch Ophthalmol 1988; 106:1378.
  64. 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.
  65. Goh KY, Schatz NJ, Glaser JS. Optic nerve sheath fenestration for pseudotumor cerebri. J Neuroophthalmol 1997; 17:86.
  66. Kelman SE, Heaps R, Wolf A, Elman MJ. Optic nerve decompression surgery improves visual function in patients with pseudotumor cerebri. Neurosurgery 1992; 30:391.
  67. Kelman SE, Sergott RC, Cioffi GA, et al. Modified optic nerve decompression in patients with functioning lumboperitoneal shunts and progressive visual loss. Ophthalmology 1991; 98:1449.
  68. Lee AG, Patrinely JR, Edmond JC. Optic nerve sheath decompression in pediatric pseudotumor cerebri. Ophthalmic Surg Lasers 1998; 29:514.
  69. Sergott RC, Savino PJ, Bosley TM. Modified optic nerve sheath decompression provides long-term visual improvement for pseudotumor cerebri. Arch Ophthalmol 1988; 106:1384.
  70. Spoor TC, McHenry JG. Long-term effectiveness of optic nerve sheath decompression for pseudotumor cerebri. Arch Ophthalmol 1993; 111:632.
  71. Plotnik JL, Kosmorsky GS. Operative complications of optic nerve sheath decompression. Ophthalmology 1993; 100:683.
  72. Hupp SL, Glaser JS, Frazier-Byrne S. Optic nerve sheath decompression. Review of 17 cases. Arch Ophthalmol 1987; 105:386.
  73. Banta JT, Farris BK. Pseudotumor cerebri and optic nerve sheath decompression. Ophthalmology 2000; 107:1907.
  74. Feldon SE. Visual outcomes comparing surgical techniques for management of severe idiopathic intracranial hypertension. Neurosurg Focus 2007; 23:E6.
  75. Spoor TC, Ramocki JM, Madion MP, Wilkinson MJ. Treatment of pseudotumor cerebri by primary and secondary optic nerve sheath decompression. Am J Ophthalmol 1991; 112:177.
  76. 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.
  77. Thuente DD, Buckley EG. Pediatric optic nerve sheath decompression. Ophthalmology 2005; 112:724.
  78. Brodsky MC, Rettele GA. Protracted postsurgical blindness with visual recovery following optic nerve sheath fenestration. Arch Ophthalmol 1997; 115:1473.
  79. Rizzo JF 3rd, Lessell S. Choroidal infarction after optic nerve sheath fenestration. Ophthalmology 1994; 101:1622.
  80. Mauriello JA Jr, Shaderowfsky P, Gizzi M, Frohman L. Management of visual loss after optic nerve sheath decompression in patients with pseudotumor cerebri. Ophthalmology 1995; 102:441.
  81. Burgett RA, Purvin VA, Kawasaki A. Lumboperitoneal shunting for pseudotumor cerebri. Neurology 1997; 49:734.
  82. Eggenberger ER, Miller NR, Vitale S. Lumboperitoneal shunt for the treatment of pseudotumor cerebri. Neurology 1996; 46:1524.
  83. Johnston I, Besser M, Morgan MK. Cerebrospinal fluid diversion in the treatment of benign intracranial hypertension. J Neurosurg 1988; 69:195.
  84. Chumas PD, Kulkarni AV, Drake JM, et al. Lumboperitoneal shunting: a retrospective study in the pediatric population. Neurosurgery 1993; 32:376.
  85. Rosenberg ML, Corbett JJ, Smith C, et al. Cerebrospinal fluid diversion procedures in pseudotumor cerebri. Neurology 1993; 43:1071.
  86. Lundar T, Nornes H. Pseudotumour cerebri-neurosurgical considerations. Acta Neurochir Suppl (Wien) 1990; 51:366.
  87. 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.
  88. Liu GT, Volpe NJ, Schatz NJ, et al. Severe sudden visual loss caused by pseudotumor cerebri and lumboperitoneal shunt failure. Am J Ophthalmol 1996; 122:129.
  89. Chumas PD, Armstrong DC, Drake JM, et al. Tonsillar herniation: the rule rather than the exception after lumboperitoneal shunting in the pediatric population. J Neurosurg 1993; 78:568.
  90. Sell JJ, Rupp FW, Orrison WW Jr. Iatrogenically induced intracranial hypotension syndrome. AJR Am J Roentgenol 1995; 165:1513.
  91. Padmanabhan R, Crompton D, Burn D, Birchall D. Acquired Chiari 1 malformation and syringomyelia following lumboperitoneal shunting for pseudotumour cerebri. J Neurol Neurosurg Psychiatry 2005; 76:298.
  92. Suri A, Pandey P, Mehta VS. Subarachnoid hemorrhage and intracereebral hematoma following lumboperitoneal shunt for pseudotumor cerebri: a rare complication. Neurol India 2002; 50:508.
  93. Bynke G, Zemack G, Bynke H, Romner B. Ventriculoperitoneal shunting for idiopathic intracranial hypertension. Neurology 2004; 63:1314.
  94. Maher CO, Garrity JA, Meyer FB. Refractory idiopathic intracranial hypertension treated with stereotactically planned ventriculoperitoneal shunt placement. Neurosurg Focus 2001; 10:E1.
  95. Woodworth GF, McGirt MJ, Elfert P, et al. Frameless stereotactic ventricular shunt placement for idiopathic intracranial hypertension. Stereotact Funct Neurosurg 2005; 83:12.
  96. 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.
  97. 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.
  98. 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.
  99. 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 Am J Neuroradiol 2011; 32:1408.
  100. Puffer RC, Mustafa W, Lanzino G. Venous sinus stenting for idiopathic intracranial hypertension: a review of the literature. J Neurointerv Surg 2013; 5:483.
  101. Friedman DI. Cerebral venous pressure, intra-abdominal pressure, and dural venous sinus stenting in idiopathic intracranial hypertension. J Neuroophthalmol 2006; 26:61.