Idiopathic intracranial hypertension (pseudotumor cerebri): Clinical features and diagnosis

INTRODUCTION

Idiopathic intracranial hypertension (IIH) is also commonly 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]. IIH primarily affects women of childbearing age who are overweight.

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.

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

CLINICAL PRESENTATION

A typical presentation of idiopathic intracranial hypertension IIH is that of an obese woman of childbearing age who complains of headaches and is found to have papilledema on funduscopic examination. Other potential high risk groups are discussed separately. (See "Idiopathic intracranial hypertension (pseudotumor cerebri): Epidemiology and pathogenesis", section on 'Risk factors' and "Idiopathic intracranial hypertension (pseudotumor cerebri): Epidemiology and pathogenesis", section on 'Associated conditions'.)

Symptoms — In one case series, the most common symptoms of idiopathic intracranial hypertension (IIH) were [2]:

                   

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: Oct 2014. | This topic last updated: Oct 2, 2014.
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 ©2014 UpToDate, Inc.
References
Top
  1. Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension. Neurology 2002; 59:1492.
  2. Wall M, George D. Idiopathic intracranial hypertension. A prospective study of 50 patients. Brain 1991; 114 ( Pt 1A):155.
  3. 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.
  4. Wall M. The headache profile of idiopathic intracranial hypertension. Cephalalgia 1990; 10:331.
  5. Lessell S. Pediatric pseudotumor cerebri (idiopathic intracranial hypertension). Surv Ophthalmol 1992; 37:155.
  6. Friedman DI, Rausch EA. Headache diagnoses in patients with treated idiopathic intracranial hypertension. Neurology 2002; 58:1551.
  7. Mathew NT, Ravishankar K, Sanin LC. Coexistence of migraine and idiopathic intracranial hypertension without papilledema. Neurology 1996; 46:1226.
  8. 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.
  9. Quattrone A, Bono F, Fera F, Lavano A. Isolated unilateral abducens palsy in idiopathic intracranial hypertension without papilledema. Eur J Neurol 2006; 13:670.
  10. Torun N, Sharpe J. Pseudotumor cerebri mimicking Foster Kennedy syndrome. Neuroophthalmology 1996; 16:55.
  11. Cinciripini GS, Donahue S, Borchert MS. Idiopathic intracranial hypertension in prepubertal pediatric patients: characteristics, treatment, and outcome. Am J Ophthalmol 1999; 127:178.
  12. Lim M, Kurian M, Penn A, et al. Visual failure without headache in idiopathic intracranial hypertension. Arch Dis Child 2005; 90:206.
  13. Bruce BB, Kedar S, Van Stavern GP, et al. Idiopathic intracranial hypertension in men. Neurology 2009; 72:304.
  14. 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.
  15. Thambisetty M, Lavin PJ, Newman NJ, Biousse V. Fulminant idiopathic intracranial hypertension. Neurology 2007; 68:229.
  16. Liu GT, Glaser JS, Schatz NJ. High-dose methylprednisolone and acetazolamide for visual loss in pseudotumor cerebri. Am J Ophthalmol 1994; 118:88.
  17. Kidron D, Pomeranz S. Malignant pseudotumor cerebri. Report of two cases. J Neurosurg 1989; 71:443.
  18. Fraser CL, Biousse V, Newman NJ. Minocycline-induced fulminant intracranial hypertension. Arch Neurol 2012; 69:1067.
  19. Rudnick E, Sismanis A. Pulsatile tinnitus and spontaneous cerebrospinal fluid rhinorrhea: indicators of benign intracranial hypertension syndrome. Otol Neurotol 2005; 26:166.
  20. Sismanis A, Butts FM, Hughes GB. Objective tinnitus in benign intracranial hypertension: an update. Laryngoscope 1990; 100:33.
  21. Chari C, Rao NS. Benign intracranial hypertension--its unusual manifestations. Headache 1991; 31:599.
  22. Wall M, White WN 2nd. Asymmetric papilledema in idiopathic intracranial hypertension: prospective interocular comparison of sensory visual function. Invest Ophthalmol Vis Sci 1998; 39:134.
  23. Greenfield DS, Wanichwecharungruang B, Liebmann JM, Ritch R. Pseudotumor cerebri appearing with unilateral papilledema after trabeculectomy. Arch Ophthalmol 1997; 115:423.
  24. Saito J, Kami M, Taniguchi F, et al. Unilateral papilledema after bone marrow transplantation. Bone Marrow Transplant 1999; 23:963.
  25. Acheson JF. Idiopathic intracranial hypertension and visual function. Br Med Bull 2006; 79-80:233.
  26. Krishna R, Kosmorsky GS, Wright KW. Pseudotumor cerebri sine papilledema with unilateral sixth nerve palsy. J Neuroophthalmol 1998; 18:53.
  27. Marcelis J, Silberstein SD. Idiopathic intracranial hypertension without papilledema. Arch Neurol 1991; 48:392.
  28. Vieira DS, Masruha MR, Gonçalves AL, et al. Idiopathic intracranial hypertension with and without papilloedema in a consecutive series of patients with chronic migraine. Cephalalgia 2008; 28:609.
  29. Beri S, Gosalakkal JA, Hussain N, et al. Idiopathic intracranial hypertension without papilledema. Pediatr Neurol 2010; 42:56.
  30. Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology 2013; 81:1159.
  31. Galetta SL, Balcer LJ. All choked up about the pseudotumor cerebri syndrome. Neurology 2013; 81:1112.
  32. Soler D, Cox T, Bullock P, et al. Diagnosis and management of benign intracranial hypertension. Arch Dis Child 1998; 78:89.
  33. Rangwala LM, Liu GT. Pediatric idiopathic intracranial hypertension. Surv Ophthalmol 2007; 52:597.
  34. 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.
  35. Salman MS, Kirkham FJ, MacGregor DL. Idiopathic "benign" intracranial hypertension: case series and review. J Child Neurol 2001; 16:465.
  36. Warman R. Management of pseudotumor cerebri in children. Int Pediatr 2000; 15:147.
  37. Kunte H, Schmidt F, Kronenberg G, et al. Olfactory dysfunction in patients with idiopathic intracranial hypertension. Neurology 2013; 81:379.
  38. Schmidt C, Wiener E, Hoffmann J, et al. Structural olfactory nerve changes in patients suffering from idiopathic intracranial hypertension. PLoS One 2012; 7:e35221.
  39. Capobianco DJ, Brazis PW, Cheshire WP. Idiopathic intracranial hypertension and seventh nerve palsy. Headache 1997; 37:286.
  40. Tan H. Bilateral oculomotor palsy secondary to pseudotumor cerebri. Pediatr Neurol 2010; 42:141.
  41. Speer C, Pearlman J, Phillips PH, et al. Fourth cranial nerve palsy in pediatric patients with pseudotumor cerebri. Am J Ophthalmol 1999; 127:236.
  42. Arsava EM, Uluc K, Nurlu G, Kansu T. Electrophysiological evidence of trigeminal neuropathy in pseudotumor cerebri. J Neurol 2002; 249:1601.
  43. Selky AK, Dobyns WB, Yee RD. Idiopathic intracranial hypertension and facial diplegia. Neurology 1994; 44:357.
  44. Dorman PJ, Campbell MJ, Maw AR. Hearing loss as a false localising sign in raised intracranial pressure. J Neurol Neurosurg Psychiatry 1995; 58:516.
  45. Malomo AO, Idowu OE, Shokunbi MT, et al. Non-operative management of benign intracranial hypertension presenting with complete visual loss and deafness. Pediatr Neurosurg 2006; 42:62.
  46. Biousse V, Rucker JC, Vignal C, et al. Anemia and papilledema. Am J Ophthalmol 2003; 135:437.
  47. Friedman DI. Papilledema and pseudotumor cerebri. Ophthalmol Clin North Am 2001; 14:129.
  48. Lin A, Foroozan R, Danesh-Meyer HV, et al. Occurrence of cerebral venous sinus thrombosis in patients with presumed idiopathic intracranial hypertension. Ophthalmology 2006; 113:2281.
  49. Brodsky MC, Vaphiades M. Magnetic resonance imaging in pseudotumor cerebri. Ophthalmology 1998; 105:1686.
  50. Gibby WA, Cohen MS, Goldberg HI, Sergott RC. Pseudotumor cerebri: CT findings and correlation with vision loss. AJR Am J Roentgenol 1993; 160:143.
  51. Jacobson DM, Karanjia PN, Olson KA, Warner JJ. Computed tomography ventricular size has no predictive value in diagnosing pseudotumor cerebri. Neurology 1990; 40:1454.
  52. Manfré L, Lagalla R, Mangiameli A, et al. Idiopathic intracranial hypertension: orbital MRI. Neuroradiology 1995; 37:459.
  53. Yuh WT, Zhu M, Taoka T, et al. MR imaging of pituitary morphology in idiopathic intracranial hypertension. J Magn Reson Imaging 2000; 12:808.
  54. Degnan AJ, Levy LM. Pseudotumor cerebri: brief review of clinical syndrome and imaging findings. AJNR Am J Neuroradiol 2011; 32:1986.
  55. Hoffmann J, Huppertz HJ, Schmidt C, et al. Morphometric and volumetric MRI changes in idiopathic intracranial hypertension. Cephalalgia 2013; 33:1075.
  56. Agid R, Farb RI, Willinsky RA, et al. Idiopathic intracranial hypertension: the validity of cross-sectional neuroimaging signs. Neuroradiology 2006; 48:521.
  57. Maralani PJ, Hassanlou M, Torres C, et al. Accuracy of brain imaging in the diagnosis of idiopathic intracranial hypertension. Clin Radiol 2012; 67:656.
  58. Ridha MA, Saindane AM, Bruce BB, et al. MRI findings of elevated intracranial pressure in cerebral venous thrombosis versus idiopathic intracranial hypertension with transverse sinus stenosis. Neuroophthalmology 2013; 37:1.
  59. Degnan AJ, Levy LM. Narrowing of Meckel's cave and cavernous sinus and enlargement of the optic nerve sheath in Pseudotumor Cerebri. J Comput Assist Tomogr 2011; 35:308.
  60. Butros SR, Goncalves LF, Thompson D, et al. Imaging features of idiopathic intracranial hypertension, including a new finding: widening of the foramen ovale. Acta Radiol 2012; 53:682.
  61. Banik R, Lin D, Miller NR. Prevalence of Chiari I malformation and cerebellar ectopia in patients with pseudotumor cerebri. J Neurol Sci 2006; 247:71.
  62. Kelly LP, Saindane AM, Bruce BB, et al. Does bilateral transverse cerebral venous sinus stenosis exist in patients without increased intracranial pressure? Clin Neurol Neurosurg 2013; 115:1215.
  63. Farb RI, Vanek I, Scott JN, et al. Idiopathic intracranial hypertension: the prevalence and morphology of sinovenous stenosis. Neurology 2003; 60:1418.
  64. Riggeal BD, Bruce BB, Saindane AM, et al. Clinical course of idiopathic intracranial hypertension with transverse sinus stenosis. Neurology 2013; 80:289.
  65. Avery RA, Shah SS, Licht DJ, et al. Reference range for cerebrospinal fluid opening pressure in children. N Engl J Med 2010; 363:891.
  66. Spence JD, Amacher AL, Willis NR. Benign intracranial hypertension without papilledema: role of 24-hour cerebrospinal fluid pressure monitoring in diagnosis and management. Neurosurgery 1980; 7:326.
  67. Torbey MT, Geocadin RG, Razumovsky AY, et al. Utility of CSF pressure monitoring to identify idiopathic intracranial hypertension without papilledema in patients with chronic daily headache. Cephalalgia 2004; 24:495.
  68. Whiteley W, Al-Shahi R, Warlow CP, et al. CSF opening pressure: reference interval and the effect of body mass index. Neurology 2006; 67:1690.
  69. Corbett JJ, Mehta MP. Cerebrospinal fluid pressure in normal obese subjects and patients with pseudotumor cerebri. Neurology 1983; 33:1386.
  70. Bono F, Lupo MR, Serra P, et al. Obesity does not induce abnormal CSF pressure in subjects with normal cerebral MR venography. Neurology 2002; 59:1641.
  71. Avery RA, Licht DJ, Shah SS, et al. CSF opening pressure in children with optic nerve head edema. Neurology 2011; 76:1658.
  72. Aylward SC. Pediatric idiopathic intracranial hypertension: a need for clarification. Pediatr Neurol 2013; 49:303.
  73. Wall M, George D. Visual loss in pseudotumor cerebri. Incidence and defects related to visual field strategy. Arch Neurol 1987; 44:170.
  74. Rowe FJ, Sarkies NJ. Assessment of visual function in idiopathic intracranial hypertension: a prospective study. Eye (Lond) 1998; 12 ( Pt 1):111.
  75. Wall M. Sensory visual testing in idiopathic intracranial hypertension: measures sensitive to change. Neurology 1990; 40:1859.
  76. Galvin JA, Van Stavern GP. Clinical characterization of idiopathic intracranial hypertension at the Detroit Medical Center. J Neurol Sci 2004; 223:157.
  77. Biousse V, Ameri A, Bousser MG. Isolated intracranial hypertension as the only sign of cerebral venous thrombosis. Neurology 1999; 53:1537.
  78. Leker RR, Steiner I. Features of dural sinus thrombosis simulating pseudotumor cerebri. Eur J Neurol 1999; 6:601.
  79. Sylaja PN, Ahsan Moosa NV, Radhakrishnan K, et al. Differential diagnosis of patients with intracranial sinus venous thrombosis related isolated intracranial hypertension from those with idiopathic intracranial hypertension. J Neurol Sci 2003; 215:9.
  80. Subramaniam RM, Tress BM, King JO, et al. Transverse sinus septum: a new aetiology of idiopathic intracranial hypertension? Australas Radiol 2004; 48:114.
  81. Angeli SI, Sato Y, Gantz BJ. Glomus jugulare tumors masquerading as benign intracranial hypertension. Arch Otolaryngol Head Neck Surg 1994; 120:1277.
  82. Jicha GA, Suarez GA. Pseudotumor cerebri reversed by cardiac septal defect repair. Neurology 2003; 60:2016.
  83. Kiers L, King JO. Increased intracranial pressure following bilateral neck dissection and radiotherapy. Aust N Z J Surg 1991; 61:459.
  84. Lam BL, Schatz NJ, Glaser JS, Bowen BC. Pseudotumor cerebri from cranial venous obstruction. Ophthalmology 1992; 99:706.
  85. Siatkowski RM, Vilar NF, Sternau L, Coin CG. Blindness from bad bones. Surv Ophthalmol 1999; 43:487.
  86. Fuentes S, Metellus P, Levrier O, et al. Depressed skull fracture overlying the superior sagittal sinus causing benign intracranial hypertension. Description of two cases and review of the literature. Br J Neurosurg 2005; 19:438.
  87. Adelman JU. Headaches and papilledema secondary to dural arteriovenous malformation. Headache 1998; 38:621.
  88. Chimowitz MI, Little JR, Awad IA, et al. Intracranial hypertension associated with unruptured cerebral arteriovenous malformations. Ann Neurol 1990; 27:474.
  89. Cockerell OC, Lai HM, Ross-Russell RW. Pseudotumour cerebri associated with arteriovenous malformations. Postgrad Med J 1993; 69:637.
  90. Cognard C, Casasco A, Toevi M, et al. Dural arteriovenous fistulas as a cause of intracranial hypertension due to impairment of cranial venous outflow. J Neurol Neurosurg Psychiatry 1998; 65:308.
  91. David CA, Peerless SJ. Pseudotumor syndrome resulting from a cerebral arteriovenous malformation: case report. Neurosurgery 1995; 36:588.
  92. Lee, A, Hayman, L, Alpert, J, et al. Occult cerebral vascular causes of pseudotumor cerebri. Neuroophthalmology 1999; 21:157.
  93. Martin TJ, Bell DA, Wilson JA. Papilledema in a man with an "occult" dural arteriovenous malformation. J Neuroophthalmol 1998; 18:49.
  94. Rosenfeld JV, Widaa HA, Adams CB. Cerebral arteriovenous malformation causing benign intracranial hypertension--case report. Neurol Med Chir (Tokyo) 1991; 31:523.
  95. Silberstein P, Kottos P, Worner C, et al. Dural arteriovenous fistulae causing pseudotumour cerebri syndrome in an elderly man. J Clin Neurosci 2003; 10:242.
  96. Vorstman EB, Niemann DB, Molyneux AJ, Pike MG. Benign intracranial hypertension associated with arteriovenous malformation. Dev Med Child Neurol 2002; 44:133.
  97. Tehindrazanarivelo A, Evrard S, Schaison M, et al. Prospective study of cerebral sinus venous thrombosis in patients presenting with benign intracranial hypertension. Cerebrovasc Dis 1992; 2:22.