Semin Neurol 2015; 35(05): 527-538
DOI: 10.1055/s-0035-1563569
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Update on the Diagnosis and Treatment of Idiopathic Intracranial Hypertension

Samuel Bidot
1   Department of Ophthalmology, Fondation Ophtalmologique Adolphe de Rothschild, Paris, France
2   Department of Ophthalmology, Centre Hospitalier National des Quinze-Vingts, Paris, France
,
Beau B. Bruce
3   Department of Ophthalmology, Emory University School of Medicine, Atlanta, Georgia
4   Department of Neurology, Emory University School of Medicine, Atlanta, Georgia
5   Department of Epidemiology, Rollins School of Public Health and Laney Graduate School, Emory University, Atlanta, Georgia
› Author Affiliations
Further Information

Publication History

Publication Date:
06 October 2015 (online)

Abstract

Idiopathic intracranial hypertension (IIH) is a rare disorder occurring more frequently in obese women of childbearing age, resulting in increased intracranial pressure (ICP) from an unknown cause. Recent advances in epidemiology, imaging, and treatment have provided a better understanding of IIH in recent years, with better identification of visual risk factors and atypical forms of IIH, including fulminant IIH and spontaneous cerebrospinal fluid leaks, and a randomized clinical trial providing the rationale for the use of acetazolamide. In addition, a revised version of the modified Dandy criteria for the diagnosis of IIH was suggested in 2013, with better definition of IIH in adults and children; however, controversy regarding nomenclature has precluded its acceptance among IIH experts. Finally, questions regarding the best surgical strategy, the indications for venous sinus stenting, and the diagnostic role of the radiologic findings commonly seen in IIH have remained unanswered.

 
  • References

  • 1 Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension. Neurology 2002; 59 (10) 1492-1495
  • 2 Dandy WE. Intracranial pressure without brain tumor: diagnosis and treatment. Ann Surg 1937; 106 (4) 492-513
  • 3 Wall M, Corbett JJ. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology 2014; 83 (2) 198-199
  • 4 Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology 2013; 81 (13) 1159-1165
  • 5 Wall M. The headache profile of idiopathic intracranial hypertension. Cephalalgia 1990; 10 (6) 331-335
  • 6 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 (6) 693-701
  • 7 Wall M, George D. Idiopathic intracranial hypertension. A prospective study of 50 patients. Brain 1991; 114 (Pt 1A): 155-180
  • 8 Headache Classification Committee of the International Headache Society (IHS). The International Classification of Headache Disorders, 3rd edition (beta version). Cephalalgia 2013; 33 (9) 629-808
  • 9 Wall M. Idiopathic intracranial hypertension. Neurol Clin 2010; 28 (3) 593-617
  • 10 Bruce BB, Newman NJ, Biousse V. Ophthalmoparesis in idiopathic intracranial hypertension. Am J Ophthalmol 2006; 142 (5) 878-880
  • 11 Schlosser RJ, Woodworth BA, Wilensky EM, Grady MS, Bolger WE. Spontaneous cerebrospinal fluid leaks: a variant of benign intracranial hypertension. Ann Otol Rhinol Laryngol 2006; 115 (7) 495-500
  • 12 Schievink WI. Spontaneous spinal cerebrospinal fluid leaks. Cephalalgia 2008; 28 (12) 1345-1356
  • 13 Wang EW, Vandergrift III WA, Schlosser RJ. Spontaneous CSF leaks. Otolaryngol Clin North Am 2011; 44 (4) 845-856 , vii
  • 14 Hayreh SS. Optic disc edema in raised intracranial pressure. V. Pathogenesis. Arch Ophthalmol 1977; 95 (9) 1553-1565
  • 15 Wall M, White II WN. Asymmetric papilledema in idiopathic intracranial hypertension: prospective interocular comparison of sensory visual function. Invest Ophthalmol Vis Sci 1998; 39 (1) 134-142
  • 16 Bidot S, Bruce BB, Saindane AM, Newman NJ, Biousse V. Asymmetric papilledema in idiopathic intracranial hypertension. J Neuroophthalmol 2015; 35 (1) 31-36
  • 17 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 (6) 705-711
  • 18 Hayreh SS. Pathogenesis of oedema of the optic disc (papilloedema). A preliminary report. Br J Ophthalmol 1964; 48: 522-543
  • 19 Wall M. The importance of visual field testing in idiopathic intracranial hypertension. Continuum (Minneap Minn) 2014; 20 (4 Neuro-ophthalmology): 1067-1074
  • 20 Hoye III VJ, Berrocal AM, Hedges III TR, Amaro-Quireza ML. Optical coherence tomography demonstrates subretinal macular edema from papilledema. Arch Ophthalmol 2001; 119 (9) 1287-1290
  • 21 Karam EZ, Hedges TR. Optical coherence tomography of the retinal nerve fibre layer in mild papilloedema and pseudopapilloedema. Br J Ophthalmol 2005; 89 (3) 294-298
  • 22 Menke MN, Feke GT, Trempe CL. OCT measurements in patients with optic disc edema. Invest Ophthalmol Vis Sci 2005; 46 (10) 3807-3811
  • 23 Kupersmith MJ, Sibony P, Mandel G, Durbin M, Kardon RH. Optical coherence tomography of the swollen optic nerve head: deformation of the peripapillary retinal pigment epithelium layer in papilledema. Invest Ophthalmol Vis Sci 2011; 52 (9) 6558-6564
  • 24 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
  • 25 Jinkins JR, Athale S, Xiong L, Yuh WT, Rothman MI, Nguyen PT. MR of optic papilla protrusion in patients with high intracranial pressure. AJNR Am J Neuroradiol 1996; 17 (4) 665-668
  • 26 Skau M, Sander B, Milea D, Jensen R. Disease activity in idiopathic intracranial hypertension: a 3-month follow-up study. J Neurol 2011; 258 (2) 277-283
  • 27 Rebolleda G, Muñoz-Negrete FJ. Follow-up of mild papilledema in idiopathic intracranial hypertension with optical coherence tomography. Invest Ophthalmol Vis Sci 2009; 50 (11) 5197-5200
  • 28 Kulkarni KM, Pasol J, Rosa PR, Lam BL. Differentiating mild papilledema and buried optic nerve head drusen using spectral domain optical coherence tomography. Ophthalmology 2014; 121 (4) 959-963
  • 29 Johnson LN, Diehl ML, Hamm CW, Sommerville DN, Petroski GF. Differentiating optic disc edema from optic nerve head drusen on optical coherence tomography. Arch Ophthalmol 2009; 127 (1) 45-49
  • 30 Lee KM, Woo SJ, Hwang JM. Differentiation of optic nerve head drusen and optic disc edema with spectral-domain optical coherence tomography. Ophthalmology 2011; 118 (5) 971-977
  • 31 Sarac O, Tasci YY, Gurdal C, Can I. Differentiation of optic disc edema from optic nerve head drusen with spectral-domain optical coherence tomography. J Neuroophthalmol 2012; 32 (3) 207-211
  • 32 Merchant KY, Su D, Park SC , et al. Enhanced depth imaging optical coherence tomography of optic nerve head drusen. Ophthalmology 2013; 120 (7) 1409-1414
  • 33 Wester ST, Fantes FE, Lam BL, Anderson DR, McSoley JJ, Knighton RW. Characteristics of optic nerve head drusen on optical coherence tomography images. Ophthalmic Surg Lasers Imaging 2010; 41 (1) 83-90
  • 34 Degnan AJ, Levy LM. Pseudotumor cerebri: brief review of clinical syndrome and imaging findings. AJNR Am J Neuroradiol 2011; 32 (11) 1986-1993
  • 35 Brodsky MC, Vaphiades M. Magnetic resonance imaging in pseudotumor cerebri. Ophthalmology 1998; 105 (9) 1686-1693
  • 36 Butros SR, Goncalves LF, Thompson D, Agarwal A, Lee HK. Imaging features of idiopathic intracranial hypertension, including a new finding: widening of the foramen ovale. Acta Radiol 2012; 53 (6) 682-688
  • 37 Aiken AH, Hoots JA, Saindane AM, Hudgins PA. Incidence of cerebellar tonsillar ectopia in idiopathic intracranial hypertension: a mimic of the Chiari I malformation. AJNR Am J Neuroradiol 2012; 33 (10) 1901-1906
  • 38 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) 1-6
  • 39 Lee SCM, Lueck CJ. Cerebrospinal fluid pressure in adults. J Neuroophthalmol 2014; 34 (3) 278-283
  • 40 Avery RA, Shah SS, Licht DJ , et al. Reference range for cerebrospinal fluid opening pressure in children. N Engl J Med 2010; 363 (9) 891-893
  • 41 Abel AS, Brace JR, McKinney AM , et al. Effect of patient positioning on cerebrospinal fluid opening pressure. J Neuroophthalmol 2014; 34 (3) 218-222
  • 42 Dasheiff R, Friedman DI, Liu GT, Digre KB. Ducks in a row. Neurology 2014; 82 (11) 1012
  • 43 Ball AK, Clarke CE. Idiopathic intracranial hypertension. Lancet Neurol 2006; 5 (5) 433-442
  • 44 Lamirel C, Bruce BB, Newman N, Biousse V. Choroidal infarction in fulminant idiopathic intracranial hypertension. J Neuroophthalmol 2010; 30 (2) 167-168
  • 45 Schirmer CM, Hedges III TR. Mechanisms of visual loss in papilledema. Neurosurg Focus 2007; 23 (5) E5
  • 46 Bruce BB, Kedar S, Van Stavern GP , et al. Idiopathic intracranial hypertension in men. Neurology 2009; 72 (4) 304-309
  • 47 Bruce BB, Preechawat P, Newman NJ, Lynn MJ, Biousse V. Racial differences in idiopathic intracranial hypertension. Neurology 2008; 70 (11) 861-867
  • 48 Szewka AJ, Bruce BB, Newman NJ, Biousse V. Idiopathic intracranial hypertension: relation between obesity and visual outcomes. J Neuroophthalmol 2013; 33 (1) 4-8
  • 49 Thambisetty M, Lavin PJ, Newman NJ, Biousse V. Fulminant idiopathic intracranial hypertension. Neurology 2007; 68 (3) 229-232
  • 50 Saindane AM, Bruce BB, Riggeal BD, Newman NJ, Biousse V. Association of MRI findings and visual outcome in idiopathic intracranial hypertension. AJR Am J Roentgenol 2013; 201 (2) 412-418
  • 51 Clark D, Bullock P, Hui T, Firth J. Benign intracranial hypertension: a cause of CSF rhinorrhoea. J Neurol Neurosurg Psychiatry 1994; 57 (7) 847-849
  • 52 Aaron G, Doyle J, Vaphiades MS, Riley KO, Woodworth BA. Increased intracranial pressure in spontaneous CSF leak patients is not associated with papilledema. Otolaryngol Head Neck Surg 2014; 151 (6) 1061-1066
  • 53 Pérez MA, Bialer OY, Bruce BB, Newman NJ, Biousse V. Primary spontaneous cerebrospinal fluid leaks and idiopathic intracranial hypertension. J Neuroophthalmol 2013; 33 (4) 330-337
  • 54 Rosenfeld E, Dotan G, Kimchi TJ, Kesler A. Spontaneous cerebrospinal fluid otorrhea and rhinorrhea in idiopathic intracranial hypertension patients. J Neuroophthalmol 2013; 33 (2) 113-116
  • 55 Lloyd KM, DelGaudio JM, Hudgins PA. Imaging of skull base cerebrospinal fluid leaks in adults. Radiology 2008; 248 (3) 725-736
  • 56 Seth R, Rajasekaran III K, Luong A, Benninger MS, Batra PS. Spontaneous CSF leaks: factors predictive of additional interventions. Laryngoscope 2010; 120 (11) 2141-2146
  • 57 Friedman DI, Rausch EA. Headache diagnoses in patients with treated idiopathic intracranial hypertension. Neurology 2002; 58 (10) 1551-1553
  • 58 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 (4) 635-641
  • 59 Kleinschmidt JJ, Digre KB, Hanover R. Idiopathic intracranial hypertension: relationship to depression, anxiety, and quality of life. Neurology 2000; 54 (2) 319-324
  • 60 Digre KB, Bruce BB, McDermott MP, Galetta KM, Balcer LJ, Wall M ; NORDIC Idiopathic Intracranial Hypertension Study Group. Quality of life in idiopathic intracranial hypertension at diagnosis: IIH Treatment Trial results. Neurology 2015 Jun 16; 84 (24) 2449-2456
  • 61 Biousse V, Rucker JC, Vignal C, Crassard I, Katz BJ, Newman NJ. Anemia and papilledema. Am J Ophthalmol 2003; 135 (4) 437-446
  • 62 Lee AG, Golnik K, Kardon R, Wall M, Eggenberger E, Yedavally S. Sleep apnea and intracranial hypertension in men. Ophthalmology 2002; 109 (3) 482-485
  • 63 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 (12) 2313-2317
  • 64 Jensen MD, Ryan DH, Apovian CM , et al; American College of Cardiology/American Heart Association Task Force on Practice Guidelines; Obesity Society. 2013 AHA/ACC/TOS guideline 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. J Am Coll Cardiol 2014; 63 (25 Pt B): 2985-3023
  • 65 Kupersmith MJ, Gamell L, Turbin R, Peck V, Spiegel P, Wall M. Effects of weight loss on the course of idiopathic intracranial hypertension in women. Neurology 1998; 50 (4) 1094-1098
  • 66 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
  • 67 Kruger RS, Pricolo VE, Streeter TT, Colacchio DA, Andrade UA. A bariatric surgery center of excellence: operative trends and long-term outcomes. J Am Coll Surg 2014; 218 (6) 1163-1174
  • 68 Biousse V, Bruce BB, Newman NJ. Update on the pathophysiology and management of idiopathic intracranial hypertension. J Neurol Neurosurg Psychiatry 2012; 83 (5) 488-494
  • 69 Farb RI, Vanek I, Scott JN , et al. Idiopathic intracranial hypertension: the prevalence and morphology of sinovenous stenosis. Neurology 2003; 60 (9) 1418-1424
  • 70 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 (6) 342-344
  • 71 Friedman DI. The pseudotumor cerebri syndrome. Neurol Clin 2014; 32 (2) 363-396
  • 72 Birzis L, Carter CH, Maren TH. Effects of acetazolamide on CSF pressure and electrolytes in hydrocephalus. Neurology 1958; 8 (7) 522-528
  • 73 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
  • 74 Horton JC. Acetazolamide for pseudotumor cerebri: evidence from the NORDIC trial. JAMA 2014; 311 (16) 1618-1619
  • 75 Çelebisoy N, Gökçay F, Şirin H, Akyürekli O. Treatment of idiopathic intracranial hypertension: topiramate vs acetazolamide, an open-label study. Acta Neurol Scand 2007; 116 (5) 322-327
  • 76 Spitze A, Malik A, Lee AG. Surgical and endovascular interventions in idiopathic intracranial hypertension. Curr Opin Neurol 2014; 27 (1) 69-74
  • 77 Higgins JNP, Cousins C, Owler BK, Sarkies N, Pickard JD. Idiopathic intracranial hypertension: 12 cases treated by venous sinus stenting. J Neurol Neurosurg Psychiatry 2003; 74 (12) 1662-1666
  • 78 Bussière M, Falero R, Nicolle D, Proulx A, Patel V, Pelz D. Unilateral transverse sinus stenting of patients with idiopathic intracranial hypertension. AJNR Am J Neuroradiol 2010; 31 (4) 645-650
  • 79 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 (8) 1408-1414
  • 80 Fields JD, Javedani PP, Falardeau J , et al. Dural venous sinus angioplasty and stenting for the treatment of idiopathic intracranial hypertension. J Neurointerv Surg 2013; 5 (1) 62-68
  • 81 Digre KB. Neuro-ophthalmology and pregnancy: what does a neuro-ophthalmologist need to know?. J Neuroophthalmol 2011; 31 (4) 381-387
  • 82 Kesler A, Kupferminc M. Idiopathic intracranial hypertension and pregnancy. Clin Obstet Gynecol 2013; 56 (2) 389-396
  • 83 Lee AG, Pless M, Falardeau J, Capozzoli T, Wall M, Kardon RH. The use of acetazolamide in idiopathic intracranial hypertension during pregnancy. Am J Ophthalmol 2005; 139 (5) 855-859
  • 84 Falardeau J, Lobb BM, Golden S, Maxfield SD, Tanne E. The use of acetazolamide during pregnancy in intracranial hypertension patients. J Neuroophthalmol 2013; 33 (1) 9-12
  • 85 Ko MW, Liu GT. Pediatric idiopathic intracranial hypertension (pseudotumor cerebri). Horm Res Paediatr 2010; 74 (6) 381-389