Subscribe to RSS
Please copy the URL and add it into your RSS Feed Reader.
https://www.thieme-connect.de/rss/thieme/en/10.1055-s-00000071.xml
Semin Neurol 2019; 39(06): 682-691
DOI: 10.1055/s-0039-1698744
DOI: 10.1055/s-0039-1698744
Review Article
Update on the Diagnosis and Treatment of Idiopathic Intracranial Hypertension
Further Information
Publication History
Publication Date:
17 December 2019 (online)
Abstract
Advances in ophthalmic diagnostics and results of interventional clinical trials are shifting diagnosis and management of idiopathic intracranial hypertension (IIH) to be more technology- and evidence-based. In this article, the evidence supporting current diagnostic criteria, evaluation, and medical and surgical management of IIH are reviewed.
-
References
- 1 Quincke HI. Über meningitis serosa. Sammlung klinischer Vortrag. Neue Folge, Nr. 67 [Innere Medicin, Nr. 23] 1893; 23: 655-694
- 2 Dandy WE. Intracranial pressure without brain tumor: diagnosis and treatment. Ann Surg 1937; 106 (04) 492-513
- 3 Foley J. Benign forms of intracranial hypertension; toxic and otitic hydrocephalus. Brain 1955; 78 (01) 1-41
- 4 Corbett JJ, Thompson HS. The rational management of idiopathic intracranial hypertension. Arch Neurol 1989; 46 (10) 1049-1051
- 5 Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology 2013; 81 (13) 1159-1165
- 6 Wall M, Corbett JJ. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology 2014; 83 (02) 198-199
- 7 Mollan SP, Davies B, Silver NC. , et al. Idiopathic intracranial hypertension: consensus guidelines on management. J Neurol Neurosurg Psychiatry 2018; 89 (10) 1088-1100
- 8 Rowe FJ, Sarkies NJ. The relationship between obesity and idiopathic intracranial hypertension. Int J Obes Relat Metab Disord 1999; 23 (01) 54-59
- 9 Markey KA, Mollan SP, Jensen RH, Sinclair AJ. Understanding idiopathic intracranial hypertension: mechanisms, management, and future directions. Lancet Neurol 2016; 15 (01) 78-91
- 10 Szewka AJ, Bruce BB, Newman NJ, Biousse V. Idiopathic intracranial hypertension: relation between obesity and visual outcomes. J Neuroophthalmol 2013; 33 (01) 4-8
- 11 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 (04) 635-641
- 12 Wall M, McDermott MP, Kieburtz KD. , et al; NORDIC Idiopathic Intracranial Hypertension Study Group Writing Committee. 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 (16) 1641-1651
- 13 Friedman DI, McDermott MP, Kieburtz K. , et al; NORDIC IIHTT Study Group. The idiopathic intracranial hypertension treatment trial: design considerations and methods. J Neuroophthalmol 2014; 34 (02) 107-117
- 14 Wall M, Kupersmith MJ, Kieburtz KD. , et al; NORDIC Idiopathic Intracranial Hypertension Study Group. The idiopathic intracranial hypertension treatment trial: clinical profile at baseline. JAMA Neurol 2014; 71 (06) 693-701
- 15 Thambisetty M, Lavin PJ, Newman NJ, Biousse V. Fulminant idiopathic intracranial hypertension. Neurology 2007; 68 (03) 229-232
- 16 Friedman DI, Quiros PA, Subramanian PS. , et al; NORDIC IIHTT Study Group. Headache in idiopathic intracranial hypertension: findings from the idiopathic intracranial hypertension treatment trial. Headache 2017; 57 (08) 1195-1205
- 17 Friedman DI. Headaches due to low and high intracranial pressure. Continuum (Minneap Minn) 2018; 24 (4, Headache): 1066-1091
- 18 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 (08) 461-474
- 19 Orcutt JC, Page NG, Sanders MD. Factors affecting visual loss in benign intracranial hypertension. Ophthalmology 1984; 91 (11) 1303-1312
- 20 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 (2 (Pt 1)): 239-244
- 21 Sadun AA, Currie JN, Lessell S. Transient visual obscurations with elevated optic discs. Ann Neurol 1984; 16 (04) 489-494
- 22 Kaiboriboon K, Piriyawat P, Selhorst JB. Light-induced amaurosis fugax. Am J Ophthalmol 2001; 131 (05) 674-676
- 23 Bruce BB, Newman NJ, Biousse V. Ophthalmoparesis in idiopathic intracranial hypertension. Am J Ophthalmol 2006; 142 (05) 878-880
- 24 Hofmann E, Behr R, Neumann-Haefelin T, Schwager K. Pulsatile tinnitus: imaging and differential diagnosis. Dtsch Arztebl Int 2013; 110 (26) 451-458
- 25 Bassan H, Berkner L, Stolovitch C, Kesler A. Asymptomatic idiopathic intracranial hypertension in children. Acta Neurol Scand 2008; 118 (04) 251-255
- 26 Bandyopadhyay S, Jacobson DM. Clinical features of late-onset pseudotumor cerebri fulfilling the modified dandy criteria. J Neuroophthalmol 2002; 22 (01) 9-11
- 27 Galvin JA, Van Stavern GP. Clinical characterization of idiopathic intracranial hypertension at the Detroit Medical Center. J Neurol Sci 2004; 223 (02) 157-160
- 28 Rowe FJ, Sarkies NJ. Assessment of visual function in idiopathic intracranial hypertension: a prospective study. Eye (Lond) 1998; 12 (Pt 1): 111-118
- 29 Kupersmith MJ, Sibony PA, Feldon SE, Wang JK, Garvin M, Kardon R. ; OCT Sub-Study Group for the NORDIC Idiopathic Intracranial Hypertension Treatment Trial. The effect of treatment of idiopathic intracranial hypertension on prevalence of retinal and choroidal folds. Am J Ophthalmol 2017; 176: 77-86
- 30 Sibony PA, Kupersmith MJ, Feldon SE, Wang JK, Garvin M. ; OCT Substudy Group for the NORDIC Idiopathic Intracranial Hypertension Treatment Trial. Retinal and choroidal folds in papilledema. Invest Ophthalmol Vis Sci 2015; 56 (10) 5670-5680
- 31 Jacks AS, Miller NR. Spontaneous retinal venous pulsation: aetiology and significance. J Neurol Neurosurg Psychiatry 2003; 74 (01) 7-9
- 32 Degnan AJ, Levy LM. Pseudotumor cerebri: brief review of clinical syndrome and imaging findings. AJNR Am J Neuroradiol 2011; 32 (11) 1986-1993
- 33 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 (08) 1215-1219
- 34 Ranganathan S, Lee SH, Checkver A. , et al. Magnetic resonance imaging finding of empty sella in obesity related idiopathic intracranial hypertension is associated with enlarged sella turcica. Neuroradiology 2013; 55 (08) 955-961
- 35 Corbett JJ, Mehta MP. Cerebrospinal fluid pressure in normal obese subjects and patients with pseudotumor cerebri. Neurology 1983; 33 (10) 1386-1388
- 36 Gilland O, Tourtellotte WW, O'Tauma L, Henderson WG. Normal cerebrospinal fluid pressure. J Neurosurg 1974; 40 (05) 587-593
- 37 Tourtellotte WW. A selected review of reactions of the cerebrospinal fluid to disease. In: Fields WS. , ed. Neurological Diagnostic Techniques. Springfield, IL: Charles C Thomas; 1966: 25-26
- 38 Whiteley W, Al-Shahi R, Warlow CP, Zeidler M, Lueck CJ. CSF opening pressure: reference interval and the effect of body mass index. Neurology 2006; 67 (09) 1690-1691
- 39 Avery RA, Shah SS, Licht DJ. , et al. Reference range for cerebrospinal fluid opening pressure in children. N Engl J Med 2010; 363 (09) 891-893
- 40 Schwartz KM, Luetmer PH, Hunt CH. , et al. Position-related variability of CSF opening pressure measurements. AJNR Am J Neuroradiol 2013; 34 (04) 904-907
- 41 Abel AS, Brace JR, McKinney AM. , et al. Effect of patient positioning on cerebrospinal fluid opening pressure. J Neuroophthalmol 2014; 34 (03) 218-222
- 42 Andresen M, Hadi A, Petersen LG, Juhler M. Effect of postural changes on ICP in healthy and ill subjects. Acta Neurochir (Wien) 2015; 157 (01) 109-113
- 43 Smith JL. Whence pseudotumor cerebri?. J Clin Neuroophthalmol 1985; 5 (01) 55-56
- 44 Ferro JM, Canhão P, Stam J, Bousser MG, Barinagarrementeria F. ; ISCVT Investigators. Prognosis of cerebral vein and dural sinus thrombosis: results of the International Study on Cerebral Vein and Dural Sinus Thrombosis (ISCVT). Stroke 2004; 35 (03) 664-670
- 45 Ferro JM, Canhão P, Stam J. , et al; ISCVT Investigators. Delay in the diagnosis of cerebral vein and dural sinus thrombosis: influence on outcome. Stroke 2009; 40 (09) 3133-3138
- 46 Dinkin M, Moss HE. Should magnetic resonance venography be performed routinely in all patients undergoing evaluation for idiopathic intracranial hypertension?. J Neuroophthalmol 2015; 35 (04) 431-437
- 47 Kwon BJ, Han MH, Kang HS, Chang KH. MR imaging findings of intracranial dural arteriovenous fistulas: relations with venous drainage patterns. AJNR Am J Neuroradiol 2005; 26 (10) 2500-2507
- 48 Rekate HL. Why would a spinal tumor cause increased intracranial pressure?. J Neuroophthalmol 2002; 22 (03) 197-198
- 49 Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension. Neurology 2002; 59 (10) 1492-1495
- 50 Digre KB, Nakamoto BK, Warner JEA, Langeberg WJ, Baggaley SK, Katz BJ. A comparison of idiopathic intracranial hypertension with and without papilledema. Headache 2009; 49 (02) 185-193
- 51 Kerr NM, Chew SS, Eady EK, Gamble GD, Danesh-Meyer HV. Diagnostic accuracy of confrontation visual field tests. Neurology 2010; 74 (15) 1184-1190
- 52 Wall M. The importance of visual field testing in idiopathic intracranial hypertension. Continuum (Minneap Minn) 2014; 20 (4 Neuro-ophthalmology): 1067-1074
- 53 Hayreh SS. Optic disc edema in raised intracranial pressure. V. Pathogenesis. Arch Ophthalmol 1977; 95 (09) 1553-1565
- 54 Tso MO, Hayreh SS. Optic disc edema in raised intracranial pressure. III. A pathologic study of experimental papilledema. Arch Ophthalmol 1977; 95 (08) 1448-1457
- 55 Keltner JL, Johnson CA, Cello KE, Wall M. ; NORDIC Idiopathic Intracranial Hypertension Study Group. Baseline visual field findings in the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT). Invest Ophthalmol Vis Sci 2014; 55 (05) 3200-3207
- 56 Corbett JJ, Jacobson DM, Mauer RC, Thompson HS. Enlargement of the blind spot caused by papilledema. Am J Ophthalmol 1988; 105 (03) 261-265
- 57 Frisén L. Swelling of the optic nerve head: a staging scheme. J Neurol Neurosurg Psychiatry 1982; 45 (01) 13-18
- 58 Sinclair AJ, Burdon MA, Nightingale PG. , et al. Rating papilloedema: an evaluation of the Frisén classification in idiopathic intracranial hypertension. J Neurol 2012; 259 (07) 1406-1412
- 59 Bruce BB, Lamirel C, Biousse V. , et al. Feasibility of nonmydriatic ocular fundus photography in the emergency department: Phase I of the FOTO-ED study. Acad Emerg Med 2011; 18 (09) 928-933
- 60 Bruce BB, Biousse V, Newman NJ. Nonmydriatic ocular fundus photography in neurologic emergencies. JAMA Neurol 2015; 72 (04) 455-459
- 61 Kelly LP, Garza PS, Bruce BB, Graubart EB, Newman NJ, Biousse V. Teaching ophthalmoscopy to medical students (the TOTeMS study). Am J Ophthalmol 2013; 156 (05) 1056-1061.e10
- 62 Thulasi P, Fraser CL, Biousse V, Wright DW, Newman NJ, Bruce BB. Nonmydriatic ocular fundus photography among headache patients in an emergency department. Neurology 2013; 80 (05) 432-437
- 63 Bruce BB, Thulasi P, Fraser CL. , et al. Diagnostic accuracy and use of nonmydriatic ocular fundus photography by emergency physicians: phase II of the FOTO-ED study. Ann Emerg Med 2013; 62 (01) 28-33.e1
- 64 Fischer WS, Wall M, McDermott MP, Kupersmith MJ, Feldon SE. ; NORDIC Idiopathic Intracranial Hypertension Study Group. Photographic reading center of the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT): Methods and Baseline Results. Invest Ophthalmol Vis Sci 2015; 56 (05) 3292-3303
- 65 Biousse V, Bruce BB, Newman NJ. Ophthalmoscopy in the 21st century: The 2017 H. Houston Merritt Lecture. Neurology 2018; 90 (04) 167-175
- 66 Chen JJ, Trobe JD. Optical coherence tomography should be used routinely to monitor patients with idiopathic intracranial hypertension. J Neuroophthalmol 2016; 36 (04) 453-459
- 67 Scott CJ, Kardon RH, Lee AG, Frisén L, Wall M. Diagnosis and grading of papilledema in patients with raised intracranial pressure using optical coherence tomography vs clinical expert assessment using a clinical staging scale. Arch Ophthalmol 2010; 128 (06) 705-711
- 68 Huang-Link Y, Eleftheriou A, Yang G. , et al. Optical coherence tomography represents a sensitive and reliable tool for routine monitoring of idiopathic intracranial hypertension with and without papilledema. Eur J Neurol 2019; 26 (05) 808-e57
- 69 Chen JJ, Thurtell MJ, Longmuir RA. , et al. Causes and prognosis of visual acuity loss at the time of initial presentation in idiopathic intracranial hypertension. Invest Ophthalmol Vis Sci 2015; 56 (06) 3850-3859
- 70 Sibony P, Kupersmith MJ, Rohlf FJ. Shape analysis of the peripapillary RPE layer in papilledema and ischemic optic neuropathy. Invest Ophthalmol Vis Sci 2011; 52 (11) 7987-7995
- 71 Sheils CR, Fischer WS, Hollar RA, Blanchard LM, Feldon SE. ; NORDIC Idiopathic Intracranial Hypertension Study Group. The relationship between optic disc volume, area, and frisén score in patients with idiopathic intracranial hypertension. Am J Ophthalmol 2018; 195: 101-109
- 72 Gampa A, Vangipuram G, Shirazi Z, Moss HE. Quantitative association between peripapillary bruch's membrane shape and intracranial pressure. Invest Ophthalmol Vis Sci 2017; 58 (05) 2739-2745
- 73 Wang JK, Kardon RH, Ledolter J, Sibony PA, Kupersmith MJ, Garvin MK. ; OCT Sub-Study Committee and the NORDIC Idiopathic Intracranial Hypertension Study Group. Peripapillary retinal pigment epithelium layer shape changes from acetazolamide treatment in the idiopathic intracranial hypertension treatment trial. Invest Ophthalmol Vis Sci 2017; 58 (05) 2554-2565
- 74 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
- 75 Manfield JH, Yu KK, Efthimiou E, Darzi A, Athanasiou T, Ashrafian H. Bariatric surgery or non-surgical weight loss for idiopathic intracranial hypertension? A systematic review and comparison of meta-analyses. Obes Surg 2017; 27 (02) 513-521
- 76 Ko MW, Chang SC, Ridha MA. , et al. Weight gain and recurrence in idiopathic intracranial hypertension: a case-control study. Neurology 2011; 76 (18) 1564-1567
- 77 Weil R, Kovacs B, Miller N. , et al; NORDIC Idiopathic Intracranial Hypertension Study Group. A 6-month telephone-based weight loss intervention in overweight and obese subjects with idiopathic intracranial hypertension. Obes Sci Pract 2016; 2 (02) 95-103
- 78 Moss HE. Bariatric surgery and the neuro-ophthalmologist. J Neuroophthalmol 2016; 36 (01) 78-84
- 79 Ottridge R, Mollan SP, Botfield H. , et al. Randomised controlled trial of bariatric surgery versus a community weight loss programme for the sustained treatment of idiopathic intracranial hypertension: the Idiopathic Intracranial Hypertension Weight Trial (IIH:WT) protocol. BMJ Open 2017; 7 (09) e017426
- 80 Gücer G, Viernstein L. Long-term intracranial pressure recording in the management of pseudotumor cerebri. J Neurosurg 1978; 49 (02) 256-263
- 81 Wall M, Kupersmith MJ, Thurtell MJ, Moss HE, Moss EA, Auinger P. ; NORDIC Idiopathic Intracranial Hypertension Study Group. The longitudinal idiopathic intracranial hypertension trial: outcomes from months 6-12. Am J Ophthalmol 2017; 176: 102-107
- 82 ten Hove MW, Friedman DI, Patel AD, Irrcher I, Wall M, McDermott MP. ; NORDIC Idiopathic Intracranial Hypertension Study Group. Safety and tolerability of acetazolamide in the idiopathic intracranial hypertension treatment trial. J Neuroophthalmol 2016; 36 (01) 13-19
- 83 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 (05) 322-327
- 84 Scotton WJ, Botfield HF, Westgate CS. , et al. Topiramate is more effective than acetazolamide at lowering intracranial pressure. Cephalalgia 2019; 39 (02) 209-218
- 85 Schoeman JF. Childhood pseudotumor cerebri: clinical and intracranial pressure response to acetazolamide and furosemide treatment in a case series. J Child Neurol 1994; 9 (02) 130-134
- 86 Chandran A, Pulhorn H, McMahon C. Idiopathic intracranial hypertension VISION (venous intervention versus shunting in IIH for optic nerve disc swelling) trial: patient perspective questionnaire. Br J Neurosurg 2019; 33 (01) 71-75
- 87 Gilbert AL, Chwalisz B, Mallery R. Complications of optic nerve sheath fenestration as a treatment for idiopathic intracranial hypertension. Semin Ophthalmol 2018; 33 (01) 36-41
- 88 Feldon SE. Visual outcomes comparing surgical techniques for management of severe idiopathic intracranial hypertension. Neurosurg Focus 2007; 23 (05) E6
- 89 Hickman SJ, Raoof N, Panesar H, McMullan JM, Pepper IM, Sharrack B. Visual outcomes from shunting for idiopathic intracranial hypertension. Neuroophthalmology 2014; 38 (06) 310-319
- 90 McGirt MJ, Woodworth G, Thomas G, Miller N, Williams M, Rigamonti D. 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 (04) 627-632
- 91 Dinkin MJ, Patsalides A. Venous sinus stenting in idiopathic intracranial hypertension: results of a prospective trial. J Neuroophthalmol 2017; 37 (02) 113-121
- 92 Lippa LM, Boker J, Duke A, Amin A. A novel 3-year longitudinal pilot study of medical students' acquisition and retention of screening eye examination skills. Ophthalmology 2006; 113 (01) 133-139
- 93 Bruce BB, Lamirel C, Wright DW. , et al. Nonmydriatic ocular fundus photography in the emergency department. N Engl J Med 2011; 364 (04) 387-389
- 94 Koerner JC, Friedman DI. Inpatient and emergency service utilization in patients with idiopathic intracranial hypertension. J Neuroophthalmol 2014; 34 (03) 229-232
- 95 Mollan SP, Aguiar M, Evison F, Frew E, Sinclair AJ. The expanding burden of idiopathic intracranial hypertension. Eye (Lond) 2019; 33 (03) 478-485
- 96 Frohman LP. Neuro-ophthalmology: transitioning from old to new models of health care delivery. J Neuroophthalmol 2017; 37 (02) 206-209