Abstract
Since obesity has become an epidemic in industrialized nations, idiopathic intracranial hypertension (IIH) is now a more common neuro-ophthalmic disorder that causes visual loss and headaches. This review highlights the new diagnostic criteria for IIH and the new insights into the pathophysiologic mechanisms of IIH. Key diagnostic and monitoring techniques for papilledema include not only neuroimaging and the measurement of cerebrospinal fluid (CSF) pressure, but also perimetry, optical coherence tomography, and ocular sonography. The main findings of the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) support acetazolamide as the mainstay for medical therapy. CSF diversion procedures, endovascular venous sinus stenting, and optic nerve sheath fenestration are all surgical options when IIH is refractory to medical treatment or when it presents fulminantly. Future clinical trials comparing these procedures will help develop better paradigms in the surgical management of IIH.
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Friesner D, Rosenman R, Lobb BM, Tanne E (2011) Idiopathic intracranial hypertension in the USA: the role of obesity in establishing prevalence and healthcare costs. Obes Rev 12(5):e372–e380
Phillips PH, Repka MX, Lambert SR (1998) Pseudotumor cerebri in children. J AAPOS 2(1):33–38
Lampl Y, Eshel Y, Kessler A, Fux A, Gilad R, Boaz M et al (2002) Serum leptin level in women with idiopathic intracranial hypertension. J Neurol Neurosurg Psychiatry 72(5):642–643
Wall M, Kupersmith MJ, Kieburtz KD, Corbett JJ, Feldon SE, Friedman DI et al (2014) The idiopathic intracranial hypertension treatment trial: clinical profile at baseline. JAMA Neurol 71(6):693–701
Yri HM, Jensen RH (2015) Idiopathic intracranial hypertension: clinical nosography and field-testing of the ICHD diagnostic criteria. A case–control study. Cephalalgia 35(7):553–562
Yri HM, Ronnback C, Wegener M, Hamann S, Jensen RH (2014) The course of headache in idiopathic intracranial hypertension: a 12-month prospective follow-up study. Eur J Neurol 21(12):1458–1464
Friedman DI, Liu GT, Digre KB (2013) Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology 81(13):1159–1165
Wall M (2010) Idiopathic intracranial hypertension. Neurol Clin 28(3):593–617
Libien J, Kupersmith MJ, Blaner W, McDermott MP, Gao S, Liu Y et al (2017) Role of vitamin A metabolism in IIH: results from the idiopathic intracranial hypertension treatment trial. J Neurol Sci 372:78–84
Vieira DS, Masruha MR, Goncalves AL, Zukerman E, Senne Soares CA, Naffah-Mazzacoratti Mda G et al (2008) Idiopathic intracranial hypertension with and without papilloedema in a consecutive series of patients with chronic migraine. Cephalalgia 28(6):609–613
De Simone R, Ranieri A, Bilo L, Fiorillo C, Bonavita V (2008) Cranial neuralgias: from physiopathology to pharmacological treatment. Neurol Sci 29(Suppl 1):S69–S78
De Simone R, Ranieri A (2015) The role of intracranial hypertension in the chronification of migraine. Neurol Sci 36(Suppl 1):23–28
Maralani PJ, Hassanlou M, Torres C, Chakraborty S, Kingstone M, Patel V et al (2012) Accuracy of brain imaging in the diagnosis of idiopathic intracranial hypertension. Clin Radiol 67(7):656–663
Kyung SE, Botelho JV, Horton JC (2014) Enlargement of the sella turcica in pseudotumor cerebri. J Neurosurg 120(2):538–542
Ranganathan S, Lee SH, Checkver A, Sklar E, Lam BL, Danton GH et al (2013) Magnetic resonance imaging finding of empty sella in obesity related idiopathic intracranial hypertension is associated with enlarged sella turcica. Neuroradiology 55(8):955–961
Bidot S, Clough L, Saindane AM, Newman NJ, Biousse V, Bruce BB (2016) The Optic Canal Size Is Associated With the Severity of Papilledema and Poor Visual Function in Idiopathic Intracranial Hypertension. J Neuro-Ophthalmol: Off J N Am Neuro-Ophthalmol Soc 36(2):120–125
Butros SR, Goncalves LF, Thompson D, Agarwal A, Lee HK (2012) Imaging features of idiopathic intracranial hypertension, including a new finding: widening of the foramen ovale. Acta Radiol 53(6):682–688
Saindane AM, Bruce BB (2014) Reply to “Enlarged CSF spaces in pseudotumor cerebri”. AJR Am J Roentgenol 203(4):W459
Bialer OY, Rueda MP, Bruce BB, Newman NJ, Biousse V, Saindane AM (2014) Meningoceles in idiopathic intracranial hypertension. AJR Am J Roentgenol 202(3):608–613
Degnan AJ, Levy LM (2011) Narrowing of Meckel’s cave and cavernous sinus and enlargement of the optic nerve sheath in pseudotumor cerebri. J Comput Assist Tomogr 35(2):308–312
Farb RI, Vanek I, Scott JN, Mikulis DJ, Willinsky RA, Tomlinson G et al (2003) Idiopathic intracranial hypertension: the prevalence and morphology of sinovenous stenosis. Neurology 60(9):1418–1424
Rohr A, Bindeballe J, Riedel C, van Baalen A, Bartsch T, Doerner L et al (2012) The entire dural sinus tree is compressed in patients with idiopathic intracranial hypertension: a longitudinal, volumetric magnetic resonance imaging study. Neuroradiology 54(1):25–33
Horev A, Hallevy H, Plakht Y, Shorer Z, Wirguin I, Shelef I (2013) Changes in cerebral venous sinuses diameter after lumbar puncture in idiopathic intracranial hypertension: a prospective MRI study. J Neuroimaging 23(3):375–378
Bono F, Giliberto C, Mastrandrea C, Cristiano D, Lavano A, Fera F et al (2005) Transverse sinus stenoses persist after normalization of the CSF pressure in IIH. Neurology 65(7):1090–1093
Kimberly HH, Shah S, Marill K, Noble V (2008) Correlation of optic nerve sheath diameter with direct measurement of intracranial pressure. Acad Emerg Med 15(2):201–204
Bauerle J, Nedelmann M (2011) Sonographic assessment of the optic nerve sheath in idiopathic intracranial hypertension. J Neurol 258(11):2014–2019
Singleton J, Dagan A, Edlow JA, Hoffmann B (2015) Real-time optic nerve sheath diameter reduction measured with bedside ultrasound after therapeutic lumbar puncture in a patient with idiopathic intracranial hypertension. Am J Emerg Med 33(6):860 e5–860 e7
Hassen GW, Nazeer O, Manizate F, Patel N, Kalantari H (2014) The role of bedside ultrasound in pretherapeutic and posttherapeutic lumbar puncture in patient with idiopathic intracranial hypertension. Am J Emerg Med 32(10):1298 e3–1298 e4
Hoffmann J, Huppertz HJ, Schmidt C, Kunte H, Harms L, Klingebiel R et al (2013) Morphometric and volumetric MRI changes in idiopathic intracranial hypertension. Cephalalgia 33(13):1075–1084
Rauz S, Cheung CM, Wood PJ, Coca-Prados M, Walker EA, Murray PI et al (2003) Inhibition of 11beta-hydroxysteroid dehydrogenase type 1 lowers intraocular pressure in patients with ocular hypertension. QJM 96(7):481–490
Sinclair AJ, Walker EA, Burdon MA, van Beek AP, Kema IP, Hughes BA et al (2010) Cerebrospinal fluid corticosteroid levels and cortisol metabolism in patients with idiopathic intracranial hypertension: a link between 11beta-HSD1 and intracranial pressure regulation? J Clin Endocrinol Metab 95(12):5348–5356
Boyle CD (2008) Recent advances in the discovery of 11beta-HSD1 inhibitors. Curr Opin Drug Discov Dev 11(4):495–511
Markey KA, Uldall M, Botfield H, Cato LD, Miah MA, Hassan-Smith G et al (2016) Idiopathic intracranial hypertension, hormones, and 11beta-hydroxysteroid dehydrogenases. J Pain Res 9:223–232
Mollan SP, Ali F, Hassan-Smith G, Botfield H, Friedman DI, Sinclair AJ (2016) Evolving evidence in adult idiopathic intracranial hypertension: pathophysiology and management. J Neurol Neurosurg Psychiatry 87(9):982–992
Iliff JJ, Wang M, Liao Y, Plogg BA, Peng W, Gundersen GA et al (2012) A paravascular pathway facilitates CSF flow through the brain parenchyma and the clearance of interstitial solutes, including amyloid beta. Sci Transl Med 4(147):147ra11
Orefice G, Celentano L, Scaglione M, Davoli M, Striano S (1992) Radioisotopic cisternography in benign intracranial hypertension of young obese women. A seven-case study and pathogenetic suggestions. Acta Neurol (Napoli) 14(1):39–50
Levitt MR, Albuquerque FC, Gross BA, Moon K, Jadhav AP, Ducruet AF et al (2016) Venous sinus stenting in patients without idiopathic intracranial hypertension. J Neurointerv Surg. doi:10.1136/neurintsurg-2016-012405
Subramaniam S, Fletcher WA (2016) Obesity and weight loss in idiopathic intracranial hypertension: a narrative review. J Neuroophthalmol. doi:10.1097/WNO.0000000000000448
Berdahl JP, Fleischman D, Zaydlarova J, Stinnett S, Allingham RR, Fautsch MP (2012) Body mass index has a linear relationship with cerebrospinal fluid pressure. Investig Ophthalmol Vis Sci 53(3):1422–1427
Kesler A, Kliper E, Shenkerman G, Stern N (2010) Idiopathic intracranial hypertension is associated with lower body adiposity. Ophthalmology 117(1):169–174
Ball AK, Sinclair AJ, Curnow SJ, Tomlinson JW, Burdon MA, Walker EA et al (2009) Elevated cerebrospinal fluid (CSF) leptin in idiopathic intracranial hypertension (IIH): evidence for hypothalamic leptin resistance? Clin Endocrinol (Oxf) 70(6):863–869
Klein A, Stern N, Osher E, Kliper E, Kesler A (2013) Hyperandrogenism is associated with earlier age of onset of idiopathic intracranial hypertension in women. Curr Eye Res 38(9):972–976
Kerty E, Heuser K, Indahl UG, Berg PR, Nakken S, Lien S et al (2013) Is the brain water channel aquaporin-4 a pathogenetic factor in idiopathic intracranial hypertension? Results from a combined clinical and genetic study in a Norwegian cohort. Acta Ophthalmol 91(1):88–91
Stiebel-Kalish H, Eyal S, Steiner I (2013) The role of aquaporin-1 in idiopathic and drug-induced intracranial hypertension. Med Hypotheses 81(6):1059–1062
Brettschneider J, Hartmann N, Lehmensiek V, Mogel H, Ludolph AC, Tumani H (2011) Cerebrospinal fluid markers of idiopathic intracranial hypertension: is the renin-angiotensinogen system involved? Cephalalgia 31(1):116–121
Edwards LJ, Sharrack B, Ismail A, Tench CR, Gran B, Dhungana S et al (2013) Increased levels of interleukins 2 and 17 in the cerebrospinal fluid of patients with idiopathic intracranial hypertension. Am J Clin Exp Immunol 2(3):234–244
Keltner JL, Johnson CA, Cello KE, Wall M (2014) Group NIIHS. Baseline visual field findings in the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT). Investig Ophthalmol Vis Sci 55(5):3200–3207
Wall M, Johnson CA, Cello KE, Zamba KD, McDermott MP, Keltner JL et al (2016) Visual field outcomes for the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT). Investig Ophthalmol Vis Sci 57(3):805–812
Corbett JJ, Jacobson DM, Mauer RC, Thompson HS (1988) Enlargement of the blind spot caused by papilledema. Am J Ophthalmol 105(3):261–265
Cello KE, Keltner JL, Johnson CA, Wall M, Group NIIHS (2016) Factors affecting visual field outcomes in the Idiopathic Intracranial Hypertension Treatment Trial. J Neuro-Ophthalmol: Off J N Am Neuro-Ophthalmol Soc 36(1):6–12
Kardon R (2014) Optical coherence tomography in papilledema: what am I missing? J Neuro-Ophthalmol: Off J N Am Neuro-Ophthalmol Soc 34(Suppl):S10–S17
Group OCTS-SCfNIIHS, Auinger P, Durbin M, Feldon S, Garvin M, Kardon R et al (2014) Baseline OCT measurements in the idiopathic intracranial hypertension treatment trial, part II: correlations and relationship to clinical features. Investig Ophthalmol Vis Sci 55(12):8173–8179
Kupersmith MJ, Sibony P, Mandel G, Durbin M, Kardon RH (2011) Optical coherence tomography of the swollen optic nerve head: deformation of the peripapillary retinal pigment epithelium layer in papilledema. Investig Ophthalmol Vis Sci 52(9):6558–6564
Sibony P, Kupersmith MJ, Honkanen R, Rohlf FJ, Torab-Parhiz A (2014) Effects of lowering cerebrospinal fluid pressure on the shape of the peripapillary retina in intracranial hypertension. Investig Ophthalmol Vis Sci 55(12):8223–8231
Silverman AL, Tatham AJ, Medeiros FA, Weinreb RN (2014) Assessment of optic nerve head drusen using enhanced depth imaging and swept source optical coherence tomography. J Neuro-Ophthalmol: Off J N Am Neuro-Ophthalmol Soc 34(2):198–205
Optical Coherence Tomography Substudy Committee, NORDIC Idiopathic Intracranial Hypertension Study Group (2015) Papilledema Outcomes from the Optical Coherence Tomography Substudy of the Idiopathic Intracranial Hypertension Treatment Trial. Ophthalmology 122(9):1939–1945
Chowdhury HR, Rajak S, Heath D, Brittain P (2015) Optical coherence tomography imaging of the optic nerve head pre optic and post optic nerve sheath fenestration. BMJ Case Rep. doi:10.1136/bcr-2014-204511
Monteiro ML, Afonso CL (2014) Macular thickness measurements with frequency domain-OCT for quantification of axonal loss in chronic papilledema from pseudotumor cerebri syndrome. Eye (London) 28(4):390–398
Afonso CL, Raza AS, Kreuz AC, Hokazono K, Cunha LP, Oyamada MK et al (2015) Relationship between pattern electroretinogram, frequency-domain OCT, and automated perimetry in chronic papilledema from pseudotumor cerebri syndrome. Invest Ophthalmol Vis Sci 56(6):3656–3665
Goldhagen BE, Bhatti MT, Srinivasan PP, Chiu SJ, Farsiu S, El-Dairi MA (2015) Retinal atrophy in eyes with resolved papilledema detected by optical coherence tomography. J Neuro-Ophthalmol: Off J N Am Neuro-Ophthalmol Soc 35(2):122–126
Ohle R, McIsaac SM, Woo MY, Perry JJ (2015) Sonography of the optic nerve sheath diameter for detection of raised intracranial pressure compared to computed tomography: a systematic review and meta-analysis. J Ultrasound Med 34(7):1285–1294
Bagnato F, Good J (2016) The use of antiepileptics in migraine prophylaxis. Headache 56(3):603–615
Friedman DI, McDermott MP, Kieburtz K, Kupersmith M, Stoutenburg A, Keltner JL et al (2014) The idiopathic intracranial hypertension treatment trial: design considerations and methods. J Neuro-Ophthalmol: Off J N Am Neuro-Ophthalmol Soc 34(2):107–117
Kupersmith MJ, Gamell L, Turbin R, Peck V, Spiegel P, Wall M (1998) Effects of weight loss on the course of idiopathic intracranial hypertension in women. Neurology 50(4):1094–1098
Levy J, Yagev R, Petrova A, Lifshitz T (2006) Topiramate-induced bilateral angle-closure glaucoma. Can J Ophthalmol 41(2):221–225
Giuliari GP, Banda RM, Vann VR, Gonzalez VH, McMillin RB (2008) Closed-angle glaucoma after topiramate therapy for migraine in a patient with undiagnosed pseudotumor cerebri. Can J Ophthalmol 43(3):371
Alore PL, Jay WM, Macken MP (2006) Topiramate, pseudotumor cerebri, weight-loss and glaucoma: an ophthalmologic perspective. Semin Ophthalmol 21(1):15–17
Celebisoy N, Gokcay F, Sirin H, Akyurekli O (2007) Treatment of idiopathic intracranial hypertension: topiramate vs acetazolamide, an open-label study. Acta Neurol Scand 116(5):322–327
Mathew NT, Ravishankar K, Sanin LC (1996) Coexistence of migraine and idiopathic intracranial hypertension without papilledema. Neurology 46(5):1226–1230
Yri HM, Wegener M, Sander B, Jensen R (2012) Idiopathic intracranial hypertension is not benign: a long-term outcome study. J Neurol 259(5):886–894
Satti SR, Leishangthem L, Chaudry MI (2015) Meta-analysis of CSF diversion procedures and dural venous sinus stenting in the setting of medically refractory idiopathic intracranial hypertension. AJNR Am J Neuroradiol 36(10):1899–1904
Brodsky MC, Rettele GA (1998) Protracted postsurgical blindness with visual recovery following optic nerve sheath fenestration. Arch Ophthalmol 116(1):107–109
Robinson ME, Moreau A, O’Meilia R, Pagteilan J, Ding K, Siatkowski RM et al (2016) The relationship between optic nerve sheath decompression failure and intracranial pressure in idiopathic intracranial hypertension. J Neuro-Ophthalmol: Off J N Am Neuro-Ophthalmol Soc 36(3):246–251
Woodworth GF, McGirt MJ, Elfert P, Sciubba DM, Rigamonti D (2005) Frameless stereotactic ventricular shunt placement for idiopathic intracranial hypertension. Stereotact Funct Neurosurg 83(1):12–16
Kandasamy J, Hayhurst C, Clark S, Jenkinson MD, Byrne P, Karabatsou K et al (2011) Electromagnetic stereotactic ventriculoperitoneal csf shunting for idiopathic intracranial hypertension: a successful step forward? World Neurosurg. 75(1):155–160 (discussion 32–3)
Alkosha HM, Zidan AS (2016) Role of lumbopleural shunt in management of idiopathic intracranial hypertension. World Neurosurg 88:113–118
Radvany MG, Solomon D, Nijjar S, Subramanian PS, Miller NR, Rigamonti D et al (2013) Visual and neurological outcomes following endovascular stenting for pseudotumor cerebri associated with transverse sinus stenosis. J Neuro-Ophthalmol: Off J N Am Neuro-Ophthalmol Soc 33(2):117–122
Dinkin MJ, Patsalides A (2016) Venous sinus stenting in idiopathic intracranial hypertension: results of a prospective trial. J Neuroophthalmol. doi:10.1097/WNO.0000000000000426
Ahmed RM, Wilkinson M, Parker GD, Thurtell MJ, Macdonald J, McCluskey PJ et al (2011) Transverse sinus stenting for idiopathic intracranial hypertension: a review of 52 patients and of model predictions. AJNR Am J Neuroradiol 32(8):1408–1414
Ahmed R, Friedman DI, Halmagyi GM (2011) Stenting of the transverse sinuses in idiopathic intracranial hypertension. J Neuro-Ophthalmol: Off J N Am Neuro-Ophthalmol Soc 31(4):374–380
American College of Cardiology/American Heart Association Task Force on Practice Guidelines OEP (2013) Executive summary: Guidelines for the management of overweight and obesity in adults: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines and the Obesity Society published by the Obesity Society and American College of Cardiology/American Heart Association Task Force on Practice Guidelines. Based on a systematic review from the The Obesity Expert Panel, 2013. Obesity (Silver Spring) 22(Suppl 2):S5–S39
Handley SC, Sun Y, Wyckoff MH, Lee HC (2015) Outcomes of extremely preterm infants after delivery room cardiopulmonary resuscitation in a population-based cohort. J Perinatol 35(5):379–383
Moss HE (2016) Bariatric Surgery and the Neuro-Ophthalmologist. J Neuro-Ophthalmol: Off J N Am Neuro-Ophthalmol Soc 36(1):78–84
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Chan, J.W. Current concepts and strategies in the diagnosis and management of idiopathic intracranial hypertension in adults. J Neurol 264, 1622–1633 (2017). https://doi.org/10.1007/s00415-017-8401-7
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DOI: https://doi.org/10.1007/s00415-017-8401-7