Abstract
Idiopathic intracranial hypertension (IIH), also known as primary pseudotumor cerebri syndrome (PTCS), is a condition of unknown etiology which affects primarily overweight, reproductive-aged women and causes increased intracranial pressure (ICP). This review discusses the recently revised diagnostic criteria for PTCS for adults and children. Additionally, the role of obesity in the epidemiology, etiology, and management of IIH as well as the current knowledge of obesity profiles and markers in IIH are reviewed. We also highlight the emerging, unifying theory of the neuroendocrine effects on the mineralocorticoid receptor to explain a possible mechanism for the increased cerebrospinal fluid production and ICP in secondary PTCS.
References
1.
Friedman DI, Liu GT, Digre KB: Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology 2013;81:1-7.
2.
Degnan AJ, Levy LM: Pseudotumor cerebri: brief review of clinical syndrome and imaging findings. AJNR Am J Neuroradiol 2011;32:1986-1993.
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;41:239-244.
4.
Soler D, Cox T, Bullock P, Calver DM, Robinson RO: Diagnosis and management of benign intracranial hypertension. Arch Dis Child 1998;78:89-94.
5.
Babikian P, Corbett J, Bell W: Idiopathic intracranial hypertension in children: the Iowa experience. J Child Neurol 1994;9:144-149.
6.
Lim M, Kurian M, Penn A, Calver D, Lin JP: Visual failure without headache in idiopathic intracranial hypertension. Arch Dis Child 2005;90:206-210.
7.
Phillips PH, Repka MX, Lambert SR: Pseudotumor cerebri in children. J AAPOS 1998;2:33-38.
8.
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.
9.
Lessell S: Pediatric pseudotumor cerebri (idiopathic intracranial hypertension). Surv Ophthalmol 1992;37:155-166.
10.
Kesler A, Fattal-Valevski A: Idiopathic intracranial hypertension in the pediatric population. J Child Neurol 2002;17:745-748.
11.
Salman MS, Kirkham FJ, MacGregor DL: Idiopathic ‘benign' intracranial hypertension: case series and review. J Child Neurol 2001;16:465-470.
12.
Salpietro V, Mankad K, Kinali M, et al: Pediatric idiopathic intracranial hypertension and the underlying endocrine-metabolic dysfunction: a pilot study. J Pediatr Endocrinol Metab 2014;27:107-115.
13.
Ko MW, Liu GT: Pediatric idiopathic intracranial hypertension (pseudotumor cerebri). Horm Res Paediatr 2010;74:381-389.
14.
Biousse V, Bruce BB, Newman NJ: Update on the pathophysiology and management of idiopathic intracranial hypertension. J Neurol Neurosurg Psychiatry 2012;83:488-494.
15.
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;22:187-194.
16.
Dandy WE: Intracranial pressure without brain tumor: diagnosis and treatment. Ann Surg 1937;106:492-513.
17.
Smith JL: Whence pseudotumor cerebri? J Clin Neuroophthalmol 1985;5:55-56.
18.
Friedman DI, Jacobson DM: Diagnostic criteria for idiopathic intracranial hypertension. Neurology 2002;59:1492-1495.
19.
Walker RW: Idiopathic intracranial hypertension: any light on the mechanism of the raised pressure? J Neurol Neurosurg Psychiatry 2001;71:1-5.
20.
Zada G, Tirosh A, Kaiser UB, Laws ER, Woodmansee WW: Cushing's disease and idiopathic intracranial hypertension: case report and review of underlying pathophysiological mechanisms. J Clin Endocrinol Metab 2010;95:4850-4854.
21.
Salpietro V, Polizzi A, Berte LF, et al: Idiopathic intracranial hypertension: a unifying neuroendocrine hypothesis through the adrenal-brain axis. Neuro Endocrinol Lett 2012;33:569-573.
22.
Khan MU, Khalid H, Salpietro V, Weber KT: Idiopathic intracranial hypertension associated with either primary or secondary aldosteronism. Am J Med Sci 2013;346:194-198.
23.
Salpietro V, Ruggieri M, Sancetta F, et al: New insights on the relationship between pseudotumor cerebri and secondary hyperaldosteronism in children. J Hypertens 2012;30:629-630.
24.
Malozowski S, Tanner LA, Wysowski D, Fleming GA: Growth hormone, insulin-like growth factor I, and benign intracranial hypertension. N Engl J Med 1993;329:665-666.
25.
Darendeliler F, Karagiannis G, Wilton P: Headache, idiopathic intracranial hypertension and slipped capital femoral epiphysis during growth hormone treatment: a safety update from the KIGS database. Horm Res 2007;68(suppl 5):41-47.
26.
Besch D, Makowski C, Steinborn MM, Bonfig W, Sadowski B: Visual loss without headache in children with pseudotumor cerebri and growth hormone treatment. Neuropediatrics 2013;44:203-207.
27.
Durcan FJ, Corbett JJ, Wall M: The incidence of pseudotumor cerebri: population studies in Iowa and Louisiana. Arch Neurol 1988;45:875-877.
28.
Craig JJ, Mulholland DA, Gibson JM: Idiopathic intracranial hypertension: incidence, presenting features and outcome in Northern Ireland (1991-1995). Ulster Med J 2001;70:31-35.
29.
Carta A, Bertuzzi F, Cologno D, Giorgi C, Montanari E, Tedesco S: Idiopathic intracranial hypertension (pseudotumor cerebri): descriptive epidemiology, clinical features, and visual outcome in Parma, Italy, 1990 to 1999. Eur J Ophthalmol 2004;14:48-54.
30.
Kesler A, Gadoth N: Epidemiology of idiopathic intracranial hypertension in Israel. J Neuroophthalmol 2001;21:12-14.
31.
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;247:474-475.
32.
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.
33.
Raoof N, Sharrack B, Pepper IM, Hickman SJ: The incidence and prevalence of idiopathic intracranial hypertension in Sheffield, UK. Eur J Neurol 2011;18:1266-1268.
34.
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;15:876-879.
35.
Liu IH, Wang AG, Yen MY: Idiopathic intracranial hypertension: clinical features in Chinese patients. Jpn J Ophthalmol 2011;55:138-142.
36.
Kapoor KG: Regarding ‘obesity may not be a risk factor for idiopathic intracranial hypertension in Asians'. Eur J Neurol 2009;16:e1, author reply e2.
37.
Tibussek D, Distelmaier F, von Kries R, Mayatepek E: Pseudotumor cerebri in childhood and adolescence - results of a Germany-wide ESPED-survey. Klin Padiatr 2013;225:81-85.
38.
Ogden CL, Carroll MD, Kit BK, Flegal KM: Prevalence of obesity and trends in body mass index among US children and adolescents, 1999-2010. JAMA 2012;307:483-490.
39.
Brara SM, Koebnick C, Porter AH, Langer-Gould A: Pediatric idiopathic intracranial hypertension and extreme childhood obesity. J Pediatr 2012;161:602-607.
40.
Kesler A, Goldhammer Y, Gadoth N: Do men with pseudomotor cerebri share the same characteristics as women? A retrospective review of 141 cases. J Neuroophthalmol 2001;21:15-17.
41.
Digre KB, Corbett JJ: Pseudotumor cerebri in men. Arch Neurol 1988;45:866-872.
42.
Bruce BB, Kedar S, Van Stavern GP, et al: Idiopathic intracranial hypertension in men. Neurology 2009;72:304-309.
43.
Fraser JA, Bruce BB, Rucker J, et al: Risk factors for idiopathic intracranial hypertension in men: a case-control study. J Neurol Sci 2010;290(1-2):86-89.
44.
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;143:635-641.
45.
Rowe FJ, Sarkies NJ: The relationship between obesity and idiopathic intracranial hypertension. Int J Obes Relat Metab Disord 1999;23:54-59.
46.
Ireland B, Corbett JJ, Wallace RB: The search for causes of idiopathic intracranial hypertension: a preliminary case-control study. Arch Neurol 1990;47:315-320.
47.
Ko MW, Chang SC, Ridha MA, et al: Weight gain and recurrence in idiopathic intracranial hypertension: a case-control study. Neurology 2011;76:1564-1567.
48.
Johnson LN, Krohel GB, Madsen RW, March GA Jr: The role of weight loss and acetazolamide in the treatment of idiopathic intracranial hypertension (pseudotumor cerebri). Ophthalmology 1998;105:2313-2317.
49.
Salpietro V, Chimenz R, Arrigo T, Ruggieri M: Pediatric idiopathic intracranial hypertension and extreme childhood obesity: a role for weight gain. J Pediatr 2013;162:1084.
50.
Newborg B: Pseudotumor cerebri treated by rice reduction diet. Arch Intern Med 1974;133:802-807.
51.
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.
52.
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;50:1094-1098.
53.
Szewka AJ, Bruce BB, Newman NJ, Biousse V: Idiopathic intracranial hypertension: relation between obesity and visual outcomes. J Neuroophthalmol 2013;33:4-8.
54.
Baldwin MK, Lobb B, Tanne E, Egan R: Weight and visual field deficits in women with idiopathic intracranial hypertension. J Womens Health (Larchmt) 2010;19:1893-1898.
55.
Sugerman HJ, Felton WL 3rd, Salvant JB Jr, Sismanis A, Kellum JM: Effects of surgically induced weight loss on idiopathic intracranial hypertension in morbid obesity. Neurology 1995;45:1655-1659.
56.
Sugerman HJ, Felton WL 3rd, Sismanis A, Kellum JM, DeMaria EJ, Sugerman EL: Gastric surgery for pseudotumor cerebri associated with severe obesity. Ann Surg 1999;229:634-640, discussion 640-642.
57.
Fridley J, Foroozan R, Sherman V, Brandt ML, Yoshor D: Bariatric surgery for the treatment of idiopathic intracranial hypertension. J Neurosurg 2011;114:34-39.
58.
Chandra V, Dutta S, Albanese CT, Shepard E, Farrales-Nguyen S, Morton J: Clinical resolution of severely symptomatic pseudotumor cerebri after gastric bypass in an adolescent. Surg Obes Relat Dis 2007;3:198-200.
59.
Kesler A, Kliper E, Shenkerman G, Stern N: Idiopathic intracranial hypertension is associated with lower body adiposity. Ophthalmology 2010;117:169-174.
60.
Ball AK, Sinclair AJ, Curnow SJ, et al: Elevated cerebrospinal fluid (CSF) leptin in idiopathic intracranial hypertension (IIH): evidence for hypothalamic leptin resistance? Clin Endocrinol (Oxf) 2009;70:863-869.
61.
Sugerman HJ, DeMaria EJ, Felton WL 3rd, Nakatsuka M, Sismanis A: Increased intra-abdominal pressure and cardiac filling pressures in obesity-associated pseudotumor cerebri. Neurology 1997;49:507-511.
62.
Sheehan JP: Hormone replacement treatment and benign intracranial hypertension. Br Med J (Clin Res Ed) 1982;284:1675-1676.
63.
Lampl Y, Eshel Y, Kessler A, et al: Serum leptin level in women with idiopathic intracranial hypertension. J Neurol Neurosurg Psychiatry 2002;72:642-643.
64.
Dhungana S, Sharrack B, Woodroofe N: Cytokines and chemokines in idiopathic intracranial hypertension. Headache 2009;49:282-285.
65.
Subramanian PS, Goldenberg-Cohen N, Shukla S, Cheskin LJ, Miller NR: Plasma ghrelin levels are normal in obese patients with idiopathic intracranial hypertension (pseudotumor cerebri). Am J Ophthalmol 2004;138:109-113.
66.
Behbehani R, Mabrook A, Abbas JM, Al-Rammah T, Mojiminiyi O, Doi SA: Is cerebrospinal fluid leptin altered in idiopathic intracranial hypertension? Clin Endocrinol (Oxf) 2010;72:851-852.
67.
Galvin JA, Van Stavern GP: Clinical characterization of idiopathic intracranial hypertension at the Detroit Medical Center. J Neurol Sci 2004;223:157-160.
68.
Celebisoy N, Secil Y, Akyurekli O: Pseudotumor cerebri: etiological factors, presenting features and prognosis in the western part of Turkey. Acta Neurol Scand 2002;106:367-370.
© 2014 S. Karger AG, Basel
2014
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.