Idiopathic intracranial hypertension: adult versus pediatric patients

Authors

  • Peter KC Leung Department of Ophthalmology, Tung Wah Eastern Hospital, Hong Kong SAR, China.
  • Jonathan CH Ho Department of Ophthalmology, Tung Wah Eastern Hospital, Hong Kong SAR, China.
  • Simon TC Ko Department of Ophthalmology, Tung Wah Eastern Hospital, Hong Kong SAR, China.

Keywords:

Headache, Idiopathic intracranial hypertension with papilledema, Obesity, Pseudotumor cerebri

Abstract

Idiopathic intracranial hypertension (IIH), also known as benign intracranial hypertension or pseudotumor cerebri, is characterized by increased intracranial pressure secondary to unknown causes. IIH affects both adults and children but predominately young obese females of child-bearing age. IIH gives rise to headache, visual disturbances, and symptoms of raised intracranial pressure. The pathophysiology of IIH remains elusive; postulations include obesity-related hormonal changes, obstruction of the central venous sinus, and hereditary causes. Diagnosing IIH is based on modified Dandy criteria. Diagnosis in children is challenging as symptoms and presentations vary; atypical and delayed presentations are not uncommon. Treatment for IIH includes oral acetazolamide or lumbar puncture; surgical treatment with shunts and optic nerve sheath fenestration is reserved for complicated and recurring cases. Treatment options for children are limited and associated with considerable risks and side effects. Vision loss is debilitating and occurs in 10% of IIH patients. A thorough and collaborative approach in managing IIH is needed. This study reviews the epidemiology, clinical presentation, diagnosis, and management of IIH in adults and pediatric patients.

References

Biousse V, Bruce BB, Newman NJ. Update on the pathophysiology and management of idiopathic intracranial hypertension. J Neurol Neurosurg Psychiatry. 2012;83:488-94.

Raoof N, Sharrack B, Pepper IM, Hickman SJ. The incidence and prevalence of idiopathic intracranial hypertension in Sheffield, UK. Eur J Neurol. 2011;18:1266-8.

Yabe I, Moriwaka F, Notoya A, Ohtaki M, Tashiro K. Incidence of idiopathic intracranial hypertension in Hokkaido, the northernmost island of Japan. J Neurol. 2000;247:474-5.

Liu IH, Wang AG, Yen MY. Idiopathic intracranial hypertension: clinical features in Chinese patients. Jpn J Ophthalmol. 2011;55:138-42.

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.

Bruce BB, Kedar S, Van Stavern GP, et al. Idiopathic intracranial hypertension in men. Neurology. 2009;72:304-9.

Mrejen S, Vignal C, Bruce BB, et al. Idiopathic intracranial hypertension: a comparison between French and North-American white patients. Rev Neurol (Paris). 2009;165:542-8.

Kim TW, Choung HK, Khwarg SI, Hwang JM, Yang HJ. Obesity may not be a risk factor for idiopathic intracranial hypertension in Asians. Eur J Neurol. 2008;15:876-9.

Ko MW, Liu GT. Pediatric idiopathic intracranial hypertension (pseudotumor cerebri). Horm Res Paediatr. 2010;74:381-9.

Lau WL, Ko CH, Cheng WW, Yau SK, Lee CY. Idiopathic intracranial hypertension in children: case report and estimation of local incidence. Hong Kong J Paediatr (new series). 2014;19:28-31.

Gordon K. Pediatric pseudotumor cerebri: descriptive epidemiology. Can J Neurol Sci. 1997;24:219-21.

Cinciripini GS, Donahue S, Borchert MS. Idiopathic intracranial hypertension in prepubertal pediatric patients: characteristics, treatment, and outcome. Am J Ophthalmol.

;127:178-82.

Sheldon CA, Paley GL, Xiao R, et al. Pediatric idiopathic intracranial hypertension: age, gender, and anthropometric features at diagnosis in a large, retrospective, multisite cohort. Ophthalmology. 2016;123:2424-31.

Kristensen B, Malm J, Markgren P, Ekstedt J. CSF hydrodynamics in superior sagittal sinus thrombosis. J Neurol Neurosurg Psychiatry. 1992;55:287-93.

Warner JE, Larson AJ, Bhosale P, et al. Retinol-binding protein and retinol analysis in cerebrospinal fluid and serum of patients with and without idiopathic intracranial hypertension. J Neuroophthalmol. 2007;27:258-62.

Tabassi A, Salmasi AH, Jalali M. Serum and CSF vitamin A concentrations in idiopathic intracranial hypertension. Neurology. 2005;64:1893-6.

Libien J, Kupersmith MJ, Blaner W, et al. Role of vitamin A metabolism in IIH: results from the idiopathic intracranial hypertension treatment trial. J Neurol Sci. 2017;372:78-84.

Wozniak SE, Gee LL, Wachtel MS, Frezza EE. Adipose tissue: the new endocrine organ? A review article. Dig Dis Sci. 2009;54:1847-56.

Ooi LY, Walker BR, Bodkin PA, Whittle IR. Idiopathic intracranial hypertension: can studies of obesity provide the key to understanding pathogenesis? Br J Neurosurg. 2008;22:187-94.

Libien J, Blaner WS. Retinol and retinol-binding protein in cerebrospinal fluid: can vitamin A take the “idiopathic” out of idiopathic intracranial hypertension? J Neuroophthalmol. 2007;27:253-7.

Rohr A, Bindeballe J, Riedel C, et al. The entire dural sinus tree is compressed in patients with idiopathic intracranial hypertension: a longitudinal, volumetric magnetic resonance imaging study. Neuroradiology. 2012;54:25-33.

Sander K, Poppert H, Etgen T, Hemmer B, Sander D. Dynamics of intracranial venous flow patterns in patients with idiopathic intracranial hypertension. Eur Neurol. 2011;66:334-8.

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.

Strydom MA, Briers N, Bosman MC, Steyn S. The anatomical basis of venographic filling defects of the transverse sinus. Clin Anat. 2010;23:153-9.

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

Albuquerque FC, Dashti SR, Hu YC, et al. Intracranial venous sinus stenting for benign intracranial hypertension: clinical indications, technique, and preliminary results. World

Neurosurg. 2011;75:648-55.

Chiu AM, Chuenkongkaew WL, Cornblath WT, et al. Minocycline treatment and pseudotumor cerebri syndrome. Am J Ophthalmol. 1998;126:116-21.

Kesler A, Goldhammer Y, Hadayer A, Pianka P. The outcome of pseudotumor cerebri induced by tetracycline therapy. Acta Neurol Scand. 2004;110:408-11.

Friedman DI. Medication-induced intracranial hypertension in dermatology. Am J Clin Dermatol. 2005;6:29-37.

Ekizoglu E, Icoz S, Tuzun E, et al. Aquaporin-4 antibodies are not present in patients with idiopathic intracranial hypertension. Cephalalgia. 2012;32:198-202.

Sinclair AJ, Viant MR, Ball AK, et al. NMR-based metabolomic analysis of cerebrospinal fluid and serum in neurological diseases--a diagnostic tool? NMR Biomed. 2010;23:123-32.

Babikian P, Corbett J, Bell W. Idiopathic intracranial hypertension in children: the Iowa experience. J Child Neurol. 1994;9:144-9.

Phillips PH, Repka MX, Lambert SR. Pseudotumor cerebri in children. J AAPOS. 1998;2:33-8.

Soler D, Cox T, Bullock P, Calver DM, Robinson RO. Diagnosis and management of benign intracranial hypertension. Arch Dis Child. 1998;78:89-94.

Wall M, Kupersmith MJ, Kieburtz KD, et al. The idiopathic intracranial hypertension treatment trial: clinical profile at baseline. JAMA Neurol. 2014;71:693-701.

Bjorner JB, Kosinski M, Ware JE Jr. Using item response theory to calibrate the Headache Impact Test (HIT) to the metric of traditional headache scales. Qual Life Res. 2003;12:981-1002.

Kosinski M, Bayliss MS, Bjorner JB, et al. A six-item shortform survey for measuring headache impact: the HIT-6. Qual Life Res. 2003;12:963-74.

Salman MS, Kirkham FJ, MacGregor DL. Idiopathic “benign” intracranial hypertension: case series and review. J Child Neurol. 2001;16:465-70.

Pollak L, Zohar E, Glovinsky Y, Huna-Baron R. Reevaluation of presentation and course of idiopathic intracranial hypertension--a large cohort comprehensive study. Acta Neurol Scand. 2013;127:406-12.

Fraser C, Plant GT. The syndrome of pseudotumour cerebri and idiopathic intracranial hypertension. Curr Opin Neurol. 2011;24:12-7.

Randhawa S, Van Stavern GP. Idiopathic intracranial hypertension (pseudotumor cerebri). Curr Opin Ophthalmol. 2008;19:445-53.

Bidot S, Bruce BB, Saindane AM, Newman NJ, Biousse V. Asymmetric papilledema in idiopathic intracranial hypertension. J Neuroophthalmol. 2015;35:31-6.

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

Frisén L. Swelling of the optic nerve head: a staging scheme. J Neurol Neurosurg Psychiatry. 1982;45:13-8.

Yau GS, Lee JW, Chan TT, Yuen CY. Unilateral papilledema and choroidal folds: an uncommon presentation of idiopathic intracranial hypertension. Surgery Curr Res. 2014;4:193.

Beri S, Gosalakkal JA, Hussain N, Balky AP, Parepalli S. Idiopathic intracranial hypertension without papilledema. Pediatr Neurol. 2010;42:56-8.

Skau M, Milea D, Sander B, Wegener M, Jensen R. OCT for optic disc evaluation in idiopathic intracranial hypertension. Graefes Arch Clin Exp Ophthalmol. 2011;249:723-30.

NORDIC Idiopathic Intracranial Hypertension Study Group Writing Committee, Wall M, McDermott MP, et al. Effect of acetazolamide on visual function in patients with idiopathic intracranial hypertension and mild visual loss: the idiopathic intracranial hypertension treatment trial. JAMA. 2014;311:1641-51.

Radhakrishnan K, Thacker AK, Bohlaga NH, Maloo JC, Gerryo SE. Epidemiology of idiopathic intracranial hypertension: a prospective and case-control study. J Neurol Sci. 1993;116:18-28.

Rush JA. Pseudotumor cerebri: clinical profile and visual outcome in 63 patients. Mayo Clin Proc. 1980;55:541-6.

Hershey AD, Powers SW, Winner P, Kabbouche MA. Pediatric Headaches in Clinical Practice. John Wiley & Sons; 2009.

Suwanwela N, Phanthumchinda K, Kaoropthum S. Headache in brain tumor: a cross-sectional study. Headache. 1994;34:435-8.

Kesler A, Fattal-Valevski A. Idiopathic intracranial hypertension in the pediatric population. J Child Neurol. 2002;17:745-8.

Youroukos S, Psychou F, Fryssiras S, Paikos P, Nicolaidou P. Idiopathic intracranial hypertension in children. J Child Neurol. 2000;15:453-7.

Standridge SM. Idiopathic intracranial hypertension in children: a review and algorithm. Pediatr Neurol. 2010;43:377-90.

Digre KB, Nakamoto BK, Warner JE, Langeberg WJ, Baggaley SK, Katz BJ. A comparison of idiopathic intracranial hypertension with and without papilledema. Headache. 2009;49:185-93.

Suzuki H, Takanashi J, Kobayashi K, Nagasawa K, Tashima K, Kohno Y. MR imaging of idiopathic intracranial hypertension. AJNR Am J Neuroradiol. 2001;22:196-9.

Rangwala LM, Liu GT. Pediatric idiopathic intracranial hypertension. Surv Ophthalmol. 2007;52:597-617.

Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension. Neurology. 2002;59:1492-5.

Friedman DI, Forman S, Levi L, Lavin PJ, Donahue S. Unusual ocular motility disturbances with increased intracranial pressure. Neurology. 1998;50:1893-6.

Corbett JJ, Mehta MP. Cerebrospinal fluid pressure in normal obese subjects and patients with pseudotumor cerebri. Neurology. 1983;33:1386-8.

Digre KB, Corbett JJ. Idiopathic intracranial hypertension (pseudotumor cerebri): a reappraisal. Neurologist. 2001;7:2-67.

Sklar FH, Beyer CW Jr, Ramanathan M, Cooper PR, Clark WK. Cerebrospinal fluid dynamics in patients with pseudotumor cerebri. Neurosurgery. 1979;5:208-16.

Agid R, Farb RI, Willinsky RA, Mikulis DJ, Tomlinson G. Idiopathic intracranial hypertension: the validity of crosssectional neuroimaging signs. Neuroradiology. 2006;48:521-7.

Lee AG, Brazis PW. Magnetic resonance venography in idiopathic pseudotumor cerebri. J Neuroophthalmol. 2000;20:12-3.

Sylaja PN, Ahsan Moosa NV, Radhakrishnan K, Sankara Sarma P, Pradeep Kumar S. Differential diagnosis of patients with intracranial sinus venous thrombosis related isolated intracranial hypertension from those with idiopathic intracranial hypertension. J Neurol Sci. 2003;215:9-12.

Leker RR, Steiner I. Features of dural sinus thrombosis simulating pseudotumor cerebri. Eur J Neurol. 1999;6:601-4.

Kotagal S. Increased Intracanial Pressure. In: Swaiman KF, Ashwal S, Ferriero DM, editors. Pediatric Neurology: Principles and Practice. Mosby Elsevier; 1999.

Welch K. The intracranial pressure in infants. J Neurosurg. 1980;52:693-9.

Lyons MK, Meyer FB. Cerebrospinal fluid physiology and the management of increased intracranial pressure. Mayo Clin Proc. 1990;65:684-707.

Johnston L, Paterson A. Benign intracranial hypertension. I. Diagnosis and prognosis. Brain. 1974;97:289-300.

Skau M, Brennum J, Gjerris F, Jensen R. What is new about idiopathic intracranial hypertension? An updated review of mechanism and treatment. Cephalalgia. 2006;26:384-99.

Wechsler B, Vidailhet M, Piette JC, et al. Cerebral venous thrombosis in Behcet’s disease: clinical study and long-term follow-up of 25 cases. Neurology. 1992;42:614-8.

Vachvanichsanong P, Dissaneewate P. Childhood systemic lupus erythematosus in Songklanagarind Hospital: a potential unique subgroup. Clin Rheumatol. 1993;12:346-9.

Padhye LV, Van Stavern GP, Sharma A, Viets R, Huecker JB, Gordon MO. Association between visual parameters and neuroimaging features of idiopathic intracranial hypertension. J Neurol Sci. 2013;332:80-5.

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.

Fridley J, Foroozan R, Sherman V, Brandt ML, Yoshor D. Bariatric surgery for the treatment of idiopathic intracranial hypertension. J Neurosurg. 2011;114:34-9.

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.

Gücer G, Viernstein L. Long-term intracranial pressure recording in the management of pseudotumor cerebri. J Neurosurg. 1978;49:256-63.

Baker RS, Baumann RJ, Buncic JR. Idiopathic intracranial hypertension (pseudotumor cerebri) in pediatric patients. Pediatr Neurol. 1989;5:5-11.

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.

Distelmaier F, Sengler U, Messing-Juenger M, Assmann B, Mayatepek E, Rosenbaum T. Pseudotumor cerebri as an important differential diagnosis of papilledema in children. Brain Dev. 2006;28:190-5.

Friedman DI. The pseudotumor cerebri syndrome. Neurol Clin. 2014;32:363-96.

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

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

Thenuwara K, Todd MM, Brian JE Jr. Effect of mannitol and furosemide on plasma osmolality and brain water. Anesthesiology. 2002;96:416-21.

Panagopoulos GN, Deftereos SN, Tagaris GA, et al. Octreotide: a therapeutic option for idiopathic intracranial hypertension. Neurol Neurophysiol Neurosci. 2007;1.

Çelebisoy N, Gökçay F, Şirin H, Akyürekli Ö. Treatment of idiopathic intracranial hypertension: topiramate vs acetazolamide, an open-label study. Acta Neurol Scand. 2007;116:322-7.

Friedman DI, Jacobson DM. Idiopathic intracranial hypertension. J Neuroophthalmol. 2004;24:138-45.

Casteels-Van Daele M, Van Geet C, Wouters C, Eggermont E. Reye syndrome revisited: a descriptive term covering a group of heterogeneous disorders. Eur J Pediatr. 2000;159:641-8.

Acheson JF, Green WT, Sanders MD. Optic nerve sheath decompression for the treatment of visual failure in chronic raised intracranial pressure. J Neurol Neurosurg Psychiatry. 1994;57:1426-9.

Goh KY, Schatz NJ, Glaser JS. Optic nerve sheath fenestration for pseudotumor cerebri. J Neuroophthalmol. 1997;17:86-91.

Mauriello JA Jr, 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.

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.

Lee AG, Patrinely JR, Edmond JC. Optic nerve sheath decompression in pediatric pseudotumor cerebri. Ophthalmic Surg Lasers. 1998;29:514-7.

Spoor TC, McHenry JG, Shin DH. Long-term results using adjunctive mitomycin C in optic nerve sheath decompression for pseudotumor cerebri. Ophthalmology. 1995;102:2024-8.

Spoor TC, McHenry JG. Long-term effectiveness of optic nerve sheath decompression for pseudotumor cerebri. Arch Ophthalmol. 1993;111:632-5.

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

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

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

Abubaker K, Ali Z, Raza K, Bolger C, Rawluk D, O’Brien D. Idiopathic intracranial hypertension: lumboperitoneal shunts versus ventriculoperitoneal shunts--case series and literature review. Br J Neurosurg. 2011;25:94-9.

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;32:1408-14.

Thurtell MJ, Wall M. Idiopathic intracranial hypertension (pseudotumor cerebri): recognition, treatment, and ongoing management. Curr Treat Options Neurol. 2013;15:1-12.

Wall M, George D. Visual loss in pseudotumor cerebri. Incidence and defects related to visual field strategy. Arch Neurol. 1987;44:170-5.

Chumas PD, Kulkarni AV, Drake JM, Hoffman HJ, Humphreys RP, Rutka JT. Lumboperitoneal shunting: a retrospective study in the pediatric population. Neurosurgery. 1993;32:376-83.

Baheti NN, Nair M, Thomas SV. Long-term visual outcome in idiopathic intracranial hypertension. Ann Indian Acad Neurol. 2011;14:19-22.

Radhakrishnan K, Ahlskog JE, Cross SA, Kurland LT, O’Fallon WM. Idiopathic intracranial hypertension (pseudotumor cerebri). Descriptive epidemiology in Rochester, Minn, 1976 to 1990. Arch Neurol. 1993;50:78-80.

Best J, Silvestri G, Burton B, Foot B, Acheson J. The incidence of blindness due to idiopathic intracranial hypertension in the UK. Open Ophthalmol J. 2013;7:26-9.

Ko MW, Chang SC, Ridha MA, et al. Weight gain and recurrence in idiopathic intracranial hypertension: a casecontrol study. Neurology. 2011;76:1564-7.

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;39:461-74.

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

Weisberg LA. Benign intracranial hypertension. Medicine (Baltimore). 1975;54:197-207.

Kesler A, Hadayer A, Goldhammer Y, Almog Y, Korczyn AD. Idiopathic intracranial hypertension: risk of recurrences. Neurology. 2004;63:1737-9.

Szewka AJ, Bruce BB, Newman NJ, Biousse V. Idiopathic intracranial hypertension: relation between obesity and visual outcomes. J Neuroophthalmol. 2013;33:4-8.

Stiebel-Kalish H, Serov I, Sella R, Chodick G, Snir M. Childhood overweight or obesity increases the risk of IIH recurrence fivefold. Int J Obes (Lond). 2014;38:1475-7.

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2018-09-01

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Leung PK, Ho JC, Ko ST. Idiopathic intracranial hypertension: adult versus pediatric patients. Hong Kong J Ophthalmol [Internet]. 2018Sep.1 [cited 2024Apr.13];22(1):9-17. Available from: https://hkjo.hk/index.php/hkjo/article/view/248

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