Cerebrospinal fluid (CSF) and idiopatic intracranial hypertension (IIH): a critical review

Authors

DOI:

https://doi.org/10.48208/HeadacheMed.2022.16

Keywords:

Headache, Pseudotumor cerebri, Idiopathic intracranial hypertension, Cerebrospinal fluid, Glymphatic system

Abstract

Pseudotumor cerebri is a syndrome that results from increased intracranial pressure. The main symptoms are headache, vision disturbances, and pulsatile tinnitus. Definitive diagnosis requires the presence of clinical and/or radiological signs of intracranial hypertension, high opening pressure on lumbar puncture (LP), and normal CSF constitution. Several studies have evaluated new contributions of CSF in the clinical evaluation and the in understanding of the pathophysiology of pseudotumor cerebri. Such studies have included the analysis of inflammatory biomarkers, adipokines, proteomic analysis, and CSF flow studies. In this review, we present the main results obtained so far and critically discuss the present status and the potential role of research involving the CSF in this condition. Based on current knowledge, it is possible to conclude that CSF research with new biomarkers has not yet provided information that can be employed in clinical practice at this moment. However, a better understanding of the constitution and dynamics of CSF circulation in patients with pseudotumor cerebri has brought some information about this condition and can potentially improve our knowledge about this condition in the future.

Downloads

Download data is not yet available.

References

Friedman DI, Liu GT and Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology 2013;81(13):1159-1165 Doi:10.1212/WNL.0b013e3182a55f17 DOI: https://doi.org/10.1212/WNL.0b013e3182a55f17

Toscano S, Lo Fermo S, Reggio E, Chisari CG, Patti F and Zappia M. An update on idiopathic intracranial hypertension in adults: a look at pathophysiology, diagnostic approach and management. J Neurol 2020;268(9):3249-3268 Doi:10.1007/s00415-020-09943-9 DOI: https://doi.org/10.1007/s00415-020-09943-9

Wall M. Update on Idiopathic Intracranial Hypertension. Neurol Clin 2017;35(1):45-57 Doi:10.1016/j.ncl.2016.08.004 DOI: https://doi.org/10.1016/j.ncl.2016.08.004

Barkatullah AF, Leishangthem L and Moss HE. MRI findings as markers of idiopathic intracranial hypertension. Curr Opin Neurol 2021;34(1):75-83 Doi:10.1097/wco.0000000000000885 DOI: https://doi.org/10.1097/WCO.0000000000000885

Raoof N and Hoffmann J. Diagnosis and treatment of idiopathic intracranial hypertension. Cephalalgia 2021;41(4):472-478 Doi:10.1177/0333102421997093 DOI: https://doi.org/10.1177/0333102421997093

Westgate CSJ, Botfield HF, Alimajstorovic Z, Yiangou A, Walsh M, Smith G, . . . Sinclair AJ. Systemic and adipocyte transcriptional and metabolic dysregulation in idiopathic intracranial hypertension. JCI Insight 2021;6(10):Doi:10.1172/jci.insight.145346 DOI: https://doi.org/10.1172/jci.insight.145346

Friedman DI. Papilledema and idiopathic intracranial hypertension. Ophtalmol Clin North 2014;20(4):857-876 Doi:10.1212/01.Con.0000453314.75261.66 DOI: https://doi.org/10.1212/01.CON.0000453314.75261.66

Avery RA, Shah SS, Licht DJ, Seiden JA, Huh JW, Boswinkel J, . . . Liu GT. Reference range for cerebrospinal fluid opening pressure in children. N Engl J Med 2010;363(9):891-893 Doi:10.1056/NEJMc1004957 DOI: https://doi.org/10.1056/NEJMc1004957

Friedman DI and Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension. Neurology 2002;59(10):1492-1495 Doi:10.1212/01.Wnl.0000029570.69134.1b DOI: https://doi.org/10.1212/01.WNL.0000029570.69134.1B

Whiteley W, Al-Shahi R, Warlow CP, Zeidler M and Lueck CJ. CSF opening pressure: Reference interval and the effect of body mass index. Neurology 2006;67(9):1690-1691 Doi:10.1212/01.wnl.0000242704.60275.e9 DOI: https://doi.org/10.1212/01.wnl.0000242704.60275.e9

Bono F, Quattrone A, Whiteley WN, Salman RAS, Lueck C and Warlow C. Csf Opening Pressure: Reference Interval and the Effect of Body Mass Index. Neurology 2007;68(17):1439-1440 Doi:10.1212/01.wnl.0000265411.84472.61 DOI: https://doi.org/10.1212/01.wnl.0000265411.84472.61

Avery RA, Licht DJ, Shah SS, Huh JW, Seiden JA, Boswinkel J, . . . Liu GT. CSF opening pressure in children with optic nerve head edema. Neurology 2011;76(19):1658-1661 Doi:10.1212/WNL.0b013e318219fb80 DOI: https://doi.org/10.1212/WNL.0b013e318219fb80

Aylward SC. Pediatric Idiopathic Intracranial Hypertension: A Need for Clarification. Pediatr Neurology 2013;49(5):303-304 Doi:10.1016/j.pediatrneurol.2013.05.019 DOI: https://doi.org/10.1016/j.pediatrneurol.2013.05.019

Berezovsky DE, Bruce BB, Vasseneix C, Peragallo JH, Newman NJ and Biousse V. Cerebrospinal fluid total protein in idiopathic intracranial hypertension. J Neurol Sci 2017;381:226-229 Doi:10.1016/j.jns.2017.08.3264 DOI: https://doi.org/10.1016/j.jns.2017.08.3264

Wall M, McDermott MP, Kieburtz KD, Corbett JJ, Feldon SE, Friedman DI, . . . Kupersmith MJ. Effect of Acetazolamide on Visual Function in Patients With Idiopathic Intracranial Hypertension and Mild Visual Loss. Jama 2014;311(16):Doi:10.1001/jama.2014.3312 DOI: https://doi.org/10.1001/jama.2014.3312

Friedman DI. Contemporary management of the pseudotumor cerebri syndrome. Exp Rev Neurother 2019;19(9):881-893 Doi:10.1080/14737175.2019.1660163 DOI: https://doi.org/10.1080/14737175.2019.1660163

Kaufman DI and Friedman DI. Should Acetazolamide Be the First-Line Treatment for Patients With Idiopathic Intracranial Hypertension? J Neuro-Ophthalmol 2017;37(2):182-186 Doi:10.1097/wno.0000000000000513 DOI: https://doi.org/10.1097/WNO.0000000000000513

Ceylan OM, Yılmaz M, Yilmaz H, Çelikay O, Köylü MT and Turan A. Neutrophil-to-lymphocyte and platelet-to-lymphocyte ratios as inflammation markers in patients with papilledema due to idiopathic intracranial hypertension. Indian J Ophthalmol 2021;69(6):1499-1505 Doi:10.4103/ijo.IJO_2030_20 DOI: https://doi.org/10.4103/ijo.IJO_2030_20

Radia T, Williams N, Agrawal P, Harman K, Weale J, Cook J and Gupta A. Multi-system inflammatory syndrome in children & adolescents (MIS-C): A systematic review of clinical features and presentation. Paediatr Resp Rev 2021;38:51-57 Doi:10.1016/j.prrv.2020.08.001 DOI: https://doi.org/10.1016/j.prrv.2020.08.001

Divya K, Indumathi C, Vikrant K and Padmanaban S. Pseudotumor cerebri complicating multisystem inflammatory syndrome in a child. J Curr Ophthalmol 2021;33(3):Doi:10.4103/joco.joco_241_20 DOI: https://doi.org/10.4103/joco.joco_241_20

Bilen NM, Sahbudak Bal Z, Yildirim Arslan S, Kanmaz S, Kurugol Z and Ozkinay F. Multisystem Inflammatory Syndrome in Children Presenting With Pseudotumor Cerebri and a Review of the Literature. Pediatr Infect Dis J 2021;40(12):e497-e500 Doi:10.1097/inf.0000000000003327 DOI: https://doi.org/10.1097/INF.0000000000003327

Sofuoğlu Aİ, Akçay N, Şevketoğlu E and Bektaş G. Pseudotumor Cerebri Syndrome as a Neurologic Involvement of Multisystem Inflammatory Syndrome in Children: A Case Report. J Trop Pediatr 2021;67(4):Doi:10.1093/tropej/fmab075 DOI: https://doi.org/10.1093/tropej/fmab075

Altıokka-Uzun G, Tüzün E, Ekizoğlu E, Ulusoy C, Yentür S, Kürtüncü M, . . . Baykan B. Oligoclonal bands and increased cytokine levels in idiopathic intracranial hypertension. Cephalalgia 2015;35(13):1153-1161 Doi:10.1177/0333102415570762 DOI: https://doi.org/10.1177/0333102415570762

Edwards LJ, Sharrack B, Ismail A, Tench CR, Gran B, Dhungana S, . . . Constantinescu CS. Increased levels of interleukins 2 and 17 in the cerebrospinal fluid of patients with idiopathic intracranial hypertension. Am J Clin Exp Immunol 2013;2(3):234-244

Dhungana S, Sharrack B and Woodroofe N. IL-1β, TNF and IP-10 in the cerebrospinal fluid and serum are not altered in patients with idiopathic intracranial hypertension compared to controls. Clin Endocrinol 2009;71(6):896-897 Doi:10.1111/j.1365-2265.2009.03593.x DOI: https://doi.org/10.1111/j.1365-2265.2009.03593.x

Fahmy EM, Rashed LA, Mostafa RH and Ismail RS. Role of tumor necrosis factor-alpha in the pathophysiology of idiopathic intracranial hypertension. Acta Neurol Scand 2021;144(5):509-516 Doi:10.1111/ane.13482 DOI: https://doi.org/10.1111/ane.13482

Zanello SB, Tadigotla V, Hurley J, Skog J, Stevens B, Calvillo E and Bershad E. Inflammatory gene expression signatures in idiopathic intracranial hypertension: possible implications in microgravity-induced ICP elevation. NPJ Microgravity 2018;4:1 Doi:10.1038/s41526-017-0036-6 DOI: https://doi.org/10.1038/s41526-017-0036-6

Mancuso P. The role of adipokines in chronic inflammation. ImmunoTargets Ther 2016; Doi:10.2147/itt.S73223 DOI: https://doi.org/10.2147/ITT.S73223

Kawai T, Autieri MV and Scalia R. Adipose tissue inflammation and metabolic dysfunction in obesity. Am J Physiol Cell 2021;320(3):C375-C391 Doi:10.1152/ajpcell.00379.2020 DOI: https://doi.org/10.1152/ajpcell.00379.2020

Obradovic M, Sudar-Milovanovic E, Soskic S, Essack M, Arya S, Stewart AJ, . . . Isenovic ER. Leptin and Obesity: Role and Clinical Implication. Front Endocrinol 2021;12:Doi:10.3389/fendo.2021.585887 DOI: https://doi.org/10.3389/fendo.2021.585887

Lu SC and Akanji AO. Leptin, Obesity, and Hypertension: A Review of Pathogenetic Mechanisms. Metab Synd Relat Disord 2020;18(9):399-405 Doi:10.1089/met.2020.0065 DOI: https://doi.org/10.1089/met.2020.0065

Ball AK, Sinclair AJ, Curnow SJ, Tomlinson JW, Burdon MA, Walker EA, . . . Rauz S. Elevated cerebrospinal fluid (CSF) leptin in idiopathic intracranial hypertension (IIH): evidence for hypothalamic leptin resistance? Clin Endocrinol (Oxf) 2009;70(6):863-869 Doi:10.1111/j.1365-2265.2008.03401.x DOI: https://doi.org/10.1111/j.1365-2265.2008.03401.x

Behbehani R, Mabrook A, Abbas JMK, Al-Rammah T, Mojiminiyi O and Doi SAR. Is cerebrospinal fluid leptin altered in idiopathic intracranial hypertension? Clin Endocrinol 2009;72(6):851-852 Doi:10.1111/j.1365-2265.2009.03722.x DOI: https://doi.org/10.1111/j.1365-2265.2009.03722.x

Samancı B, Samancı Y, Tüzün E, Altıokka-Uzun G, Ekizoğlu E, İçöz S, . . . Baykan B. Evidence for potential involvement of pro-inflammatory adipokines in the pathogenesis of idiopathic intracranial hypertension. Cephalalgia 2016;37(6):525-531 Doi:10.1177/0333102416650705 DOI: https://doi.org/10.1177/0333102416650705

Aslam B, Basit M, Nisar MA, Khurshid M and Rasool MH. Proteomics: Technologies

and Their Applications. J Chromatogr Science 2017;55(2):182-196 Doi:10.1093/chromsci/bmw167 DOI: https://doi.org/10.1093/chromsci/bmw167

Lecube A, Poca MA, Colomé N, Bech-Serra JJ, Hernández C, García-Ramírez M, . . . Simó R. Proteomic Analysis of Cerebrospinal Fluid from Obese Women with Idiopathic Intracranial Hypertension: A New Approach for Identifying New Candidates in the Pathogenesis of Obesity. J Neuroendocrinol 2012;24(6):944-952 Doi:10.1111/j.1365-2826.2012.02288.x DOI: https://doi.org/10.1111/j.1365-2826.2012.02288.x

Brettschneider J, Hartmann N, Lehmensiek V, Mogel H, Ludolph AC and Tumani H. Cerebrospinal fluid markers of idiopathic intracranial hypertension: Is the renin- angiotensinogen system involved? Cephalalgia 2010;31(1):116-121 Doi:10.1177/0333102410375726 DOI: https://doi.org/10.1177/0333102410375726

Jessen NA, Munk ASF, Lundgaard I and Nedergaard M. The Glymphatic System: A Beginner’s Guide. Neurochemical Res 2015;40(12):2583-2599 Doi:10.1007/s11064-015-1581-6 DOI: https://doi.org/10.1007/s11064-015-1581-6

Mestre H, Mori Y and Nedergaard M. The Brain’s Glymphatic System: Current Controversies. Trend Neurosci 2020;43(7):458-466 Doi:10.1016/j.tins.2020.04.003 DOI: https://doi.org/10.1016/j.tins.2020.04.003

Lenck S, Radovanovic I, Nicholson P, Hodaie M, Krings T and Mendes-Pereira V. Idiopathic intracranial hypertension. Neurology 2018;91(11):515-522 Doi:10.1212/wnl.0000000000006166 DOI: https://doi.org/10.1212/WNL.0000000000006166

Tuță S. Cerebral Venous Outflow Implications in Idiopathic Intracranial Hypertension—From Physiopathology to Treatment. Life 2022;12(6):Doi:10.3390/life12060854 DOI: https://doi.org/10.3390/life12060854

Bezerra MLS, Ferreira ACA and de Oliveira-Souza R. Pseudotumor Cerebri and Glymphatic Dysfunction. Front Neurol 2018;8 Doi:10.3389/fneur.2017.00734 DOI: https://doi.org/10.3389/fneur.2017.00734

Mondejar V and Patsalides A. The Role of Arachnoid Granulations and the Glymphatic System in the Pathophysiology of Idiopathic Intracranial Hypertension. Curr Neurol Neurosci Rep 2020;20(7):Doi:10.1007/s11910-020-01044-4 DOI: https://doi.org/10.1007/s11910-020-01044-4

Nicholson P, Kedra A, Shotar E, Bonnin S, Boch A-L, Shor N, . . . Lenck S. Idiopathic Intracranial Hypertension: Glymphedema of the Brain. J Neuro-Ophthalmol 2021;41(1):93-97 Doi:10.1097/wno.0000000000001000 DOI: https://doi.org/10.1097/WNO.0000000000001000

Jones O, Cutsforth-Gregory J, Chen J, Bhatti MT, Huston J and Brinjikji W. Idiopathic Intracranial Hypertension is Associated with a Higher Burden of Visible Cerebral Perivascular Spaces: The Glymphatic Connection. Am J Neuroradiol 2021;42(12):2160-2164 Doi:10.3174/ajnr.A7326 DOI: https://doi.org/10.3174/ajnr.A7326

Bošnjak R and Kordaš M. Circulatory effects of internal jugular vein compression: A computer simulation study. Med Biol Eng Comp 2002;40(4):423-431 Doi:10.1007/bf02345075 DOI: https://doi.org/10.1007/BF02345075

Eide PK, Hasan‐Olive MM, Hansson HA and Enger R. Increased occurrence of pathological mitochondria in astrocytic perivascular endfoot processes and neurons of idiopathic intracranial hypertension. J Neurosci Res 2020;99(2):467-480 Doi:10.1002/jnr.24743 DOI: https://doi.org/10.1002/jnr.24743

Taoka T and Naganawa S. Neurofluid Dynamics and the Glymphatic System: A Neuroimaging Perspective. Korean J Radiol 2020;21(11):Doi:10.3348/kjr.2020.0042 DOI: https://doi.org/10.3348/kjr.2020.0042

Downloads

Published

2022-10-01

How to Cite

1.
Domingues R, Vega M, Brunale F, Giafferi C, Senne C. Cerebrospinal fluid (CSF) and idiopatic intracranial hypertension (IIH): a critical review. Headache Med [Internet]. 2022 Oct. 1 [cited 2024 May 9];13(3):167-73. Available from: https://headachemedicine.com.br/index.php/hm/article/view/649

Issue

Vol. 13 No. 3 (2022)

Section

Review

License

Copyright (c) 2022 Renan Domingues, Márcio Vega, Fernando Brunale, Carlos Giafferi, Carlos Senne

Creative Commons License

This work is licensed under a Creative Commons Attribution 4.0 International License.

Most read articles by the same author(s)