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Idiopathic Intracranial Hypertension: A Venous Disease?

Part of the Springer Series in Translational Stroke Research book series (SSTSR)

Abstract

Idiopathic intracranial hypertension (IIH) is a condition characterized by a constellation of symptoms including headache, visual disturbance, tinnitus, and papilledema that affect primarily obese/young female. The pathophysiologic hallmark of the disease is a chronic elevation of intracranial pressure (opening pressure on lumbar puncture >250 cm H2O). Despite the significant morbidity associated with this condition (vision loss and debilitating headaches), the exact mechanisms leading high intracranial pressure is not known. It has been purported that cerebral venous stenosis (CVS) may play a role in the pathogenesis of idiopathic intracranial hypertension. In this chapter, we examine the evidence linking cerebral venous stenosis to IIH. From an epidemiological perspective, CVS is up to 9 times more prevalent among patients with IIH compared to their counterparts, and the cerebral venous pressure gradient across the stenotic segment by venographic manometry is higher among patients with IIH. Pathophysiological evidence for an association between CVS and IIH suggest that at least in some cases, variations in the caliber of the cerebral vein is associated with a change in cerebrospinal fluid pressure; an observation that is supported by mathematical models. In the absence of randomized clinical trials, the best evidence supporting CVS stenting are primarily provided by meta-analyses of individual studies that report a clinical benefit of the procedure albeit several shortfalls. In light of the suggested association between cerebral transverse sinus stenosis/hypoplasia and certain IIH cases, future research would need to focus on defining the clinical and radiological profile of individuals who will best benefit from CVS stenting. It is also expected that ongoing clinical trials shed more light on the association between CVS and IIH and refine the therapeutic potentials of stenting.

Keywords

  • Epidemiology
  • Treatment
  • Stenting
  • Idiopathic intracranial hypertension
  • Cerebral venous stenosis
  • Pseudotumor cerebri

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Fig. 9.1

References

  1. Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension. Neurology. 2002;59(10):1492–5.

    CrossRef  Google Scholar 

  2. Markey KA, Mollan SP, Jensen RH, Sinclair AJ. Understanding idiopathic intracranial hypertension: mechanisms, management, and future directions. Lancet Neurol. 2016;15(1):78–91.

    CrossRef  Google Scholar 

  3. Friedman DI, Jacobson DM. Idiopathic intracranial hypertension. J Neuroophthalmol. 2004;24(2):138–45.

    CrossRef  Google Scholar 

  4. Wall M. Idiopathic intracranial hypertension (pseudotumor cerebri). Curr Neurol Neurosci Rep. 2008;8(2):87–93.

    CrossRef  Google Scholar 

  5. Kesler A, Gadoth N. Epidemiology of idiopathic intracranial hypertension in Israel. J Neuroophthalmol. 2001;21(1):12–4.

    CAS  CrossRef  Google Scholar 

  6. Durcan FJ, Corbett JJ, Wall M. The incidence of pseudotumor cerebri. Population studies in Iowa and Louisiana. Arch Neurol. 1988;45(8):875–7.

    CAS  CrossRef  Google Scholar 

  7. Asensio-Sanchez VM, Merino-Angulo J, Martinez-Calvo S, Calvo MJ, Rodriguez R. [Epidemiology of pseudotumor cerebri]. Arch Soc Esp Oftalmol. 2007;82(4):219–21.

    Google Scholar 

  8. Craig JJ, Mulholland DA, Gibson JM. Idiopathic intracranial hypertension; incidence, presenting features and outcome in Northern Ireland (1991-1995). Ulster Med J. 2001;70(1):31–5.

    CAS  PubMed  PubMed Central  Google Scholar 

  9. Idiculla T, Zachariah G, Br K, Mohamood N. The incidence and prevalance of idiopathic intracranial hypertension in south Sharaqiah region, Oman. Oman J Ophthalmol. 2013;6(3):189–92.

    CrossRef  Google Scholar 

  10. 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(1):18–28.

    CAS  CrossRef  Google Scholar 

  11. 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(1):48–54.

    CAS  CrossRef  Google Scholar 

  12. Shah VA, Kardon RH, Lee AG, Corbett JJ, Wall M. Long-term follow-up of idiopathic intracranial hypertension: the Iowa experience. Neurology. 2008;70(8):634–40.

    CAS  CrossRef  Google Scholar 

  13. Ko MW, Chang SC, Ridha MA, et al. Weight gain and recurrence in idiopathic intracranial hypertension: a case-control study. Neurology. 2011;76(18):1564–7.

    CAS  CrossRef  Google Scholar 

  14. 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):635–41.

    CrossRef  Google Scholar 

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

    CAS  CrossRef  Google Scholar 

  16. Chen J, Wall M. Epidemiology and risk factors for idiopathic intracranial hypertension. Int Ophthalmol Clin. 2014;54(1):1–11.

    CrossRef  Google Scholar 

  17. Farb RI, Vanek I, Scott JN, et al. Idiopathic intracranial hypertension: the prevalence and morphology of sinovenous stenosis. Neurology. 2003;60(9):1418–24.

    CAS  CrossRef  Google Scholar 

  18. Bono F, Messina D, Giliberto C, et al. Bilateral transverse sinus stenosis predicts IIH without papilledema in patients with migraine. Neurology. 2006;67(3):419–23.

    CAS  CrossRef  Google Scholar 

  19. Bono F, Messina D, Giliberto C, et al. Bilateral transverse sinus stenosis and idiopathic intracranial hypertension without papilledema in chronic tension-type headache. J Neurol. 2008;255(6):807–12.

    CAS  CrossRef  Google Scholar 

  20. 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(8):1215–9.

    CrossRef  Google Scholar 

  21. Levitt MR, Hlubek RJ, Moon K, et al. Incidence and predictors of dural venous sinus pressure gradient in idiopathic intracranial hypertension and non-idiopathic intracranial hypertension headache patients: results from 164 cerebral venograms. J Neurosurg. 2017;126:347–53.

    CrossRef  Google Scholar 

  22. Joynt RJ, Sahs AL. Brain swelling of unknown cause. Neurology. 1956;6(11):801–3.

    CAS  PubMed  Google Scholar 

  23. Wall M, Dollar JD, Sadun AA, Kardon R. Idiopathic intracranial hypertension. Lack of histologic evidence for cerebral edema. Arch Neurol. 1995;52(2):141–5.

    CAS  CrossRef  Google Scholar 

  24. Sørensen PS, Thomsen C, Gjerris F, Henriksen O. Brain water accumulation in pseudotumour cerebri demonstrated by MR-imaging of brain water self-diffusion. Acta Neurochir Suppl (Wien). 1990;51:363–5.

    Google Scholar 

  25. Sørensen PS, Thomsen C, Gjerris F, Schmidt J, Kjaer L, Henriksen O. Increased brain water content in pseudotumour cerebri measured by magnetic resonance imaging of brain water self diffusion. Neurol Res. 1989;11(3):160–4.

    CrossRef  Google Scholar 

  26. Gideon P, Sørensen PS, Thomsen C, Ståhlberg F, Gjerris F, Henriksen O. Increased brain water self-diffusion in patients with idiopathic intracranial hypertension. AJNR Am J Neuroradiol. 1995;16(2):381–7.

    CAS  PubMed  Google Scholar 

  27. Bastin ME, Sinha S, Farrall AJ, Wardlaw JM, Whittle IR. Diffuse brain oedema in idiopathic intracranial hypertension: a quantitative magnetic resonance imaging study. J Neurol Neurosurg Psychiatry. 2003;74(12):1693–6.

    CAS  CrossRef  Google Scholar 

  28. 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):342–4.

    CrossRef  Google Scholar 

  29. Scoffings DJ, Pickard JD, Higgins JN. Resolution of transverse sinus stenoses immediately after CSF withdrawal in idiopathic intracranial hypertension. J Neurol Neurosurg Psychiatry. 2007;78(8):911–2.

    CrossRef  Google Scholar 

  30. Lee SW, Gates P, Morris P, Whan A, Riddington L. Idiopathic intracranial hypertension; immediate resolution of venous sinus “obstruction” after reducing cerebrospinal fluid pressure to<10cmH(2)O. J Clin Neurosci. 2009;16(12):1690–2.

    CrossRef  Google Scholar 

  31. Baryshnik DB, Farb RI. Changes in the appearance of venous sinuses after treatment of disordered intracranial pressure. Neurology. 2004;62(8):1445–6.

    CrossRef  Google Scholar 

  32. Bono F, Giliberto C, Mastrandrea C, et al. Transverse sinus stenoses persist after normalization of the CSF pressure in IIH. Neurology. 2005;65(7):1090–3.

    CAS  CrossRef  Google Scholar 

  33. Connor SE, Siddiqui MA, Stewart VR, O’Flynn EA. The relationship of transverse sinus stenosis to bony groove dimensions provides an insight into the aetiology of idiopathic intracranial hypertension. Neuroradiology. 2008;50(12):999–1004.

    CAS  CrossRef  Google Scholar 

  34. Stevens SA, Stimpson J, Lakin WD, Thakore NJ, Penar PL. A model for idiopathic intracranial hypertension and associated pathological ICP wave-forms. IEEE Trans Biomed Eng. 2008;55(2 Pt 1):388–98.

    CrossRef  Google Scholar 

  35. Stevens SA, Thakore NJ, Lakin WD, Penar PL, Tranmer BI. A modeling study of idiopathic intracranial hypertension: etiology and diagnosis. Neurol Res. 2007;29(8):777–86.

    CrossRef  Google Scholar 

  36. 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.

    CrossRef  Google Scholar 

  37. 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):1094–8.

    CAS  CrossRef  Google Scholar 

  38. 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(12):2313–7.

    CAS  CrossRef  Google Scholar 

  39. Committee NIIHSGW, 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(16):1641–51.

    CrossRef  Google Scholar 

  40. Hainline C, Rucker JC, Balcer LJ. Current concepts in pseudotumor cerebri. Curr Opin Neurol. 2016;29(1):84–93.

    CrossRef  Google Scholar 

  41. Iancu D, Schwarz BA. Stenting of venous sinus stenosis for medically refractory idiopathic intracranial hypertension. Ottawa: Ottawa Hospital Research Institute; 2014.

    Google Scholar 

  42. Patsalides A. Venous sinus stenting for idiopathic intracranial hypertension refractory to medical therapy (VSSIIH). New York: Weill Cornell Medical College; 2011.

    Google Scholar 

  43. Puffer RC, Mustafa W, Lanzino G. Venous sinus stenting for idiopathic intracranial hypertension: a review of the literature. J Neurointerv Surg. 2013;5(5):483–6.

    CrossRef  Google Scholar 

  44. Satti SR, Leishangthem L, Chaudry MI. Meta-analysis of CSF diversion procedures and dural venous sinus stenting in the setting of medically refractory idiopathic intracranial hypertension. AJNR Am J Neuroradiol. 2015;36(10):1899–904.

    CAS  CrossRef  Google Scholar 

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Acknowledgments

We would like to thank Drs. Samir Belagaje and Saher Khalid for their generous comments on this manuscript. Dr. Feng would like to acknowledge the grant supports from National Institute of Health (P20GM109040 and HD086844), American Heart Association (14SDG1829003) and South Carolina Clinical & Translational Research Institute (UL1 TR001450).

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Correspondence to Wuwei Feng .

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Appendix

Appendix

1.1 Search Terms

#1 “pseudotumor cerebri” OR “idiopathic intracranial hypertension” OR “benign intracranial hypertension”

#2 “venous stenosis” OR “sinus stenosis” OR “stenosis of cerebral vein”

#3 1 AND 2

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Lekoubou, A., Feng, W. (2019). Idiopathic Intracranial Hypertension: A Venous Disease?. In: , et al. Cerebral Venous System in Acute and Chronic Brain Injuries. Springer Series in Translational Stroke Research. Springer, Cham. https://doi.org/10.1007/978-3-319-96053-1_9

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  • DOI: https://doi.org/10.1007/978-3-319-96053-1_9

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