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Idiopathic Intracranial Hypertension (Pseudotumor Cerebri): Recognition, Treatment, and Ongoing Management

Current Treatment Options in Neurology volume 15, pages 1–12 (2013)Cite this article

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.

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References and Recommended Reading

Papers of particular interest, published recently, have been highlighted as: • Of importance

  1. Wall M, George D. Idiopathic intracranial hypertension: a prospective study of 50 patients. Brain. 1991;114:155–80.

    Article  PubMed  Google Scholar 

  2. 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.

    Article  PubMed  CAS  Google Scholar 

  3. Wall M. The headache profile of idiopathic intracranial hypertension. Cephalagia. 1990;10:331–5.

    Article  CAS  Google Scholar 

  4. Friedman DI, Rausch EA. Headache diagnoses in patients with treated idiopathic intracranial hypertension. Neurology. 2002;58:1551–3.

    Article  PubMed  Google Scholar 

  5. Durcan FJ, Corbett JJ, Wall M. The incidence of pseudotumor cerebri: population studies in Iowa and Louisiana. Arch Neurol. 1988;45:875–7.

    Article  PubMed  CAS  Google Scholar 

  6. 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.

    Article  PubMed  Google Scholar 

  7. 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.

    Article  PubMed  Google Scholar 

  8. Newborg B. Pseudotumor cerebri treated by rice reduction diet. Arch Intern Med. 1974;133:802–7.

    Article  PubMed  CAS  Google Scholar 

  9. 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.

    Article  PubMed  CAS  Google Scholar 

  10. 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.

    Article  PubMed  Google Scholar 

  11. Ball AK, Howman A, Wheatley K, et al. A randomised controlled trial of treatment for idiopathic intracranial hypertension. J Neurol. 2011;258:874–81.

    Article  PubMed  CAS  Google Scholar 

  12. Idiopathic Intracranial Hypertension Treatment Trial (IIHTT). Available at: http://clinicaltrials.gov/ct2/show/NCT01003639. Accessed September 2012.

  13. 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.

    Article  PubMed  CAS  Google Scholar 

  14. Falardeau J, Lobb BM, Golden S, et al. The use of acetazolamide during pregnancy in intracranial hypertension patients. J Neuroophthalmol. 2012 (in press).

  15. Tawil R, Moxley 3rd RT, Griggs RC. Acetazolamide-induced nephrolithiasis: implications for treatment of neuromuscular disorders. Neurology. 1993;43:1105–6.

    Article  PubMed  CAS  Google Scholar 

  16. Lichter PR. Reducing side effects of carbonic anhydrase inhibitors. Ophthalmology. 1981;88:266–9.

    PubMed  CAS  Google Scholar 

  17. 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.

    Article  PubMed  CAS  Google Scholar 

  18. 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.

    Article  PubMed  CAS  Google Scholar 

  19. 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.

    PubMed  CAS  Google Scholar 

  20. Neville BG, Wilson J. Benign intracranial hypertension following corticosteroid withdrawal in childhood. BMJ. 1970;3(5722):554–6.

    Article  PubMed  CAS  Google Scholar 

  21. Thambisetty M, Lavin PJ, Newman NJ, Biousse V. Fulminant idiopathic intracranial hypertension. Neurology. 2007;68:229–32.

    Article  PubMed  Google Scholar 

  22. Liu GT, Glaser JS, Schatz NJ. High-dose methylprednisolone and acetazolamide for visual loss in pseudotumor cerebri. Am J Ophthalmol. 1994;118:88–96.

    PubMed  CAS  Google Scholar 

  23. 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.

    Article  PubMed  Google Scholar 

  24. Sugerman HJ, Felton 3rd WL, Sismanis A, et al. Gastric surgery for pseudotumor cerebri associated with severe obesity. Ann Surg. 1999;229:634–40.

    Article  PubMed  CAS  Google Scholar 

  25. Fridley J, Foroozan R, Sherman V, et al. Bariatric surgery for the treatment of idiopathic intracranial hypertension. J Neurosurg. 2011;114:34–9.

    Article  PubMed  Google Scholar 

  26. Johnston I, Besser M, Morgan MK. Cerebrospinal fluid diversion in the treatment of benign intracranial hypertension. J Neurosurg. 1988;69:195–202.

    Article  PubMed  CAS  Google Scholar 

  27. Rosenberg ML, Corbett JJ, Smith C, et al. Cerebrospinal fluid diversion procedures in pseudotumor cerebri. Neurology. 1993;43:1071–2.

    Article  PubMed  CAS  Google Scholar 

  28. Eggenberger ER, Miller NR, Vitale S. Lumboperitoneal shunt for the treatment of pseudotumor cerebri. Neurology. 1996;46:1524–30.

    Article  PubMed  CAS  Google Scholar 

  29. Burgett RA, Purvin VA, Kawasaki A. Lumboperitoneal shunting for pseudotumor cerebri. Neurology. 1997;49:734–9.

    Article  PubMed  CAS  Google Scholar 

  30. Maher CO, Garrity JA, Meyer FB. Refractory idiopathic intracranial hypertension treated with stereotactically planned ventriculoperitoneal shunt placement. Neurosurg Focus. 2001;10:E1.

    Article  PubMed  CAS  Google Scholar 

  31. Bynke G, Zemack G, Bynke H, Romner B. Ventriculoperitoneal shunting for idiopathic intracranial hypertension. Neurology. 2004;63:1314–6.

    Article  PubMed  Google Scholar 

  32. 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.

    Article  PubMed  Google Scholar 

  33. 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.

    Article  PubMed  CAS  Google Scholar 

  34. 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.

    Article  PubMed  CAS  Google Scholar 

  35. Berman EL, Wirtschafter JD. Improvement of optic nerve head appearance after surgery for pseudotumor cerebri. JAMA. 1992;267:1130.

    Article  PubMed  CAS  Google Scholar 

  36. 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.

    Article  PubMed  CAS  Google Scholar 

  37. 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.

    Article  Google Scholar 

  38. 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.

    Article  PubMed  Google Scholar 

  39. Wilkes BN, Siatkowski RM. Progressive optic neuropathy in idiopathic intracranial hypertension after optic nerve sheath fenestration. J Neuroophthalmol. 2009;29:281–3.

    Article  PubMed  Google Scholar 

  40. Kaye AH, Galbraith JE, King J. Intracranial pressure following optic nerve decompression for benign intracranial hypertension. J Neurosurg. 1981;55:453–6.

    Article  PubMed  CAS  Google Scholar 

  41. 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.

    Article  PubMed  Google Scholar 

  42. Brodsky MC, Rettele GA. Protracted postsurgical blindness with visual recovery following optic nerve sheath fenestration. Arch Ophthalmol. 1998;116:107–9.

    PubMed  CAS  Google Scholar 

  43. Flynn WJ, Westfall CT, Weisman JS. Transient blindness after optic nerve sheath fenestration. Am J Ophthalmol. 1994;117:678–9.

    PubMed  CAS  Google Scholar 

  44. 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.

    PubMed  Google Scholar 

  45. Farb RI, Vanek I, Scott JN, et al. Idiopathic intracranial hypertension: the prevalence and morphology of sinovenous stenosis. Neurology. 2003;60:1418–24.

    Article  PubMed  CAS  Google Scholar 

  46. King JO, Mitchell PJ, Thomson KR, Tress BM. Cerebral venography and manometry in idiopathic intracranial hypertension. Neurology. 1995;45:2224–8.

    Article  PubMed  CAS  Google Scholar 

  47. King JO, Mitchell PJ, Thomson KR, Tress BM. Manometry combined with cervical puncture in idiopathic intracranial hypertension. Neurology. 2002;58:26–30.

    Article  PubMed  CAS  Google Scholar 

  48. Higgins JN, Pickard JD. Lateral sinus stenosis in idiopathic intracranial hypertension resolving after CSF diversion. Neurology. 2004;62:1907–8.

    Article  PubMed  Google Scholar 

  49. 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.

    Article  PubMed  Google Scholar 

  50. 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.

    Article  PubMed  Google Scholar 

  51. 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.

    Article  PubMed  CAS  Google Scholar 

  52. Owler BK, Parker G, Halmagyi GM, et al. Cranial venous outflow obstruction and pseudotumor cerebri syndrome. Adv Tech Stand Neurosurg. 2005;30:107–74.

    Article  PubMed  CAS  Google Scholar 

  53. 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.

    Article  PubMed  CAS  Google Scholar 

  54. Bussière M, Falero R, Nicolle D, et al. Unilateral transverse sinus stenting of patients with idiopathic intracranial hypertension. AJNR. 2010;31:645–50.

    Article  PubMed  Google Scholar 

  55. 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.

    Article  PubMed  CAS  Google Scholar 

  56. 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.

    Article  PubMed  CAS  Google Scholar 

  57. 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 cmH2O should not necessarily be considered abnormal.

    Article  PubMed  Google Scholar 

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Acknowledgments

Dr. Wall has received grant support from the National Institutes of Health.

Disclosure

Dr. Thurtell has received speaker honoraria from Sun Pharmaceutical Industries Ltd. and royalties from Oxford University Press.

Dr. Wall reported no potential conflicts of interest relevant to this article.

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Authors and Affiliations

  1. Department of Ophthalmology & Visual Sciences, University of Iowa, 200 Hawkins Dr PFP, Iowa City, IA, 52242, USA

    Matthew J. Thurtell MBBS, FRACP

  2. Department of Neurology, University of Iowa, 200 Hawkins Dr #2007 RCP, Iowa City, IA, 52242, USA

    Michael Wall MD

  3. Neurology Service, VA Medical Center, Iowa City, IA, USA

    Matthew J. Thurtell MBBS, FRACP & Michael Wall MD

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  1. Matthew J. Thurtell MBBS, FRACP
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Correspondence to Michael Wall MD.

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Thurtell, M.J., Wall, M. Idiopathic Intracranial Hypertension (Pseudotumor Cerebri): Recognition, Treatment, and Ongoing Management. Curr Treat Options Neurol 15, 1–12 (2013). https://doi.org/10.1007/s11940-012-0207-4

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  • DOI: https://doi.org/10.1007/s11940-012-0207-4

Keywords

  • Idiopathic intracranial hypertension
  • Treatment
  • Recognition
  • Management
  • Pseudotumor cerebri
  • Papilledema
  • Vision loss
  • Acetazolamide
  • Topiramate
  • Optic nerve sheath fenestration
  • Cerebrospinal fluid shunting