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An Up to Date Review of Pseudotumor Cerebri Syndrome

  • Headache (RB Halker, Section Editor)
  • Published:
Current Neurology and Neuroscience Reports Aims and scope Submit manuscript

Abstract

Purpose of Review

Idiopathic intracranial hypertension (IIH), pseudotumor cerebri syndrome (PTCS), and benign intracranial hypertension are all terms that have been used for a neurologic syndrome consisting of elevated intracranial pressure (ICP), headache and vision loss without mass lesion or underlying infection or malignancy. In this review article, categorization, diagnostic criteria, symptom management strategies, and disease treatment options for pseudotumor cerebri syndrome will be discussed.

Recent Findings

The Idiopathic Intracranial Hypertension Treatment Trial has now proven that acetazolamide should be the first line therapy in primary PTCS, but other treatment options exist in patients who cannot tolerate acetazolamide or in selected cases, which requires surgical intervention for PTCS which acutely threatens vision. Headache has also been shown to require focused treatment beyond therapies that lower ICP, specifically targeting coexistent primary headache disorders and medication overuse.

Summary

Advances in treatment and diagnostic modalities have improved understanding of PTCS types and their treatment. The pathophysiology of primary PTCS, however, remains incompletely understood, but continued evaluation of cerebrospinal fluid flow dynamics, aquaporins, hormones, natriuretic peptides, and the link with female gender and obesity may lead to future answers.

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References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Degnan A, Levy L. Pseudotumor cerebri: brief review of clinical syndrome and imaging findings. Am J Neuroradiol. 2011;32(11):1986–93.

    Article  PubMed  CAS  Google Scholar 

  2. Johnston I. The historical development of the pseudotumor concept. Neurosurg Focus. 2001;11(2):1–9.

    Article  Google Scholar 

  3. Friedman D, Liu G, Digre K. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology. 2013;81:1159–65.

    Article  PubMed  Google Scholar 

  4. Cleves-Bayon C. Idiopathic intracranial hypertension in children and adolescents: an update. Headache J Head Face Pain. 2017;58:485–93. https://doi.org/10.1111/head.13236.

    Article  Google Scholar 

  5. Ekizoglu E. An update on the pathophysiology of idiopathic intracranial hypertension alias pseudotumor cerebri. Ağrı. 2015; https://doi.org/10.5505/agri.2015.22599.

  6. Riggeal B, Bruce B, Saindane A, Ridha M, Kelly L, Newman N, et al. Clinical course of idiopathic intracranial hypertension with transverse sinus stenosis. Neurology. 2012;80:289–95.

    Article  PubMed  Google Scholar 

  7. Rohr A, Dörner L, Stingele R, Buhl R, Alfke K, Jansen O. Reversibility of venous sinus obstruction in idiopathic intracranial hypertension. AJNR Am J Neuroradiol. 2007;4:656–9.

    Google Scholar 

  8. • Mollan S, Ali F, Hassan-Smith G, Botfield H, Friedman D, Sinclair A. Evolving evidence in adult idiopathic intracranial hypertension: pathophysiology and management. J Neurol Neurosurg Psychiatry. 2016;87:982–92. Recent review of PTCS pathophysiology and treatment trials.

    Article  PubMed  PubMed Central  Google Scholar 

  9. Warner J, Larson A, Bhosale P, Digre K, Henley C, Alder S, 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.

    Article  PubMed  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  11. • Libien J, Kupersmith M, Blaner W, McDermott M, Gao S, Liu Y, et al. Role of vitamin A metabolism in IIH: results from the idiopathic intracranial hypertension treatment trial. J Neurol Sci. 2017;372:78–84. Vitamin A metabolite levels in patients of the Idiopathic Intracranial Hypertension Treatment Trial.

    Article  PubMed  CAS  Google Scholar 

  12. • Subramaniam S, Fletcher W. Obesity and weight loss in idiopathic intracranial hypertension. J Neuroophthalmol. 2017;37:197–205. Recent review of published literature regarding the association of PTCS and obesity.

    Article  PubMed  Google Scholar 

  13. Portelli M, Papageorgiou P. An update on idiopathic intracranial hypertension. Acta Neurochir. 2016;159(3):491–9.

    Article  PubMed  Google Scholar 

  14. Kilgore K, Lee M, Leavitt J, Mokri B, Hodge D, Frank R, et al. Re-evaluating the incidence of idiopathic intracranial hypertension in an era of increasing obesity. Ophthalmology. 2017;124:697–700.

    Article  PubMed  PubMed Central  Google Scholar 

  15. Sinclair A, Ball A, Burdon M, Clarke C, Stewart P, Curnow S, et al. Exploring the pathogenesis of IIH: an inflammatory perspective. J Neuroimmunol. 2008;201-202:212–20.

    Article  PubMed  CAS  Google Scholar 

  16. • Sinclair AJ, Markey KA, Uldall M, Cato L, Miah M, Botfield H, et al. Idiopathic intracranial hypertension, hormones, and 11beta-hydroxysteroid dehydrogenases. J Pain Res 2016;9:223–232. Review of PTCS’ correlation with hormones and obesity.

  17. Markey K, Ottridge R, Mitchell J, Rick C, Woolley R, Ives N, et al. Assessing the efficacy and safety of an 11β-Hydroxysteroid dehydrogenase type 1 inhibitor (AZD4017) in the idiopathic intracranial hypertension drug trial, IIH:DT: clinical methods and Design for a Phase II randomized controlled trial. JMIR Res Protoc. 2017;6:e181.

    Article  PubMed  PubMed Central  Google Scholar 

  18. Skau M, Goetze J, Rehfeld J, Jensen R. Natriuretic pro-peptides in idiopathic intracranial hypertension. Regul Pept. 2010;164:71–7.

    Article  PubMed  CAS  Google Scholar 

  19. Ameli P, Madan M, Chigurupati S, Yu A, Chan S, Pattisapu J Effect of acetazolamide on aquaporin-1 and fluid flow in cultured choroid plexus. Acta Neurochir Suppl 2012;113:59–64.

  20. Radhakrishnan K, Thacker A, Bohlaga N, Maloo J, Gerryo S. Epidemiology of idiopathic intracranial hypertension: a prospective and case-control study. J Neurol Sci. 1993;116(1):18–28. (epidemiology)

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  22. Kesler A, Stolovic N, Bluednikov Y, Shohat T. The incidence of idiopathic intracranial hypertension in Israel from 2005 to 2007: results of a nationwide survey. Eur J Neurol. 2014;21:1055–9.

    Article  PubMed  CAS  Google Scholar 

  23. •• Matthews Y, Dean F, Lim M, Mclachlan K, Rigby A, Solanki G, et al. Pseudotumor cerebri syndrome in childhood: incidence, clinical profile and risk factors in a national prospective population-based cohort study. Arch Dis Child. 2017;102:715–21. National prospective cohort study looking at 185 cases in the Britsh Paediatric Surveillance Unit.

    Article  PubMed  Google Scholar 

  24. •• Sundholm A, Burkill S, Sveinsson O, Piehl F, Bahmanyar S, Nilsson Remahl A. Population-based incidence and clinical characteristics of idiopathic intracranial hypertension. Acta Neurol Scand. 2017;136:427–33. (Obesity). Evaluation of the incidence of PTCS in Sweden using national patient registry.

    Article  PubMed  CAS  Google Scholar 

  25. •• Wall M, Kupersmith M, Kieburtz K, Corbett J, Feldon S, Friedman D, et al. The idiopathic intracranial hypertension treatment trial. JAMA Neurolgy. 2014;71:693. Outlines the baseline clinical characteristics of patients enrolled in the IIHTT–701.

    Article  Google Scholar 

  26. Wall M. The headache profile of idiopathic intracranial hypertension. Cephalalgia. 1990;10:331–5.

    Article  PubMed  CAS  Google Scholar 

  27. •• Friedman D, Quiros P, Subramanian P, Mejico L, Gao S, McDermott M, et al. Headache in idiopathic intracranial hypertension: findings from the idiopathic intracranial hypertension treatment trial. Headache J Head Face Pain. 2017;57:1195–205. Findings from IIHTT regarding headache and PTCS.

    Article  Google Scholar 

  28. Thambisetty M, Lavin P, Newman N, Biousse V. Fulminant idiopathic intracranial hypertension. Neurology. 2007;68:229–32.

    Article  PubMed  Google Scholar 

  29. Stevens S, Rizk H, Golnik K, Andaluz N, Samy R, Meyer T, et al. Idiopathic intracranial hypertension: contemporary review and implications for the otolaryngologist. Laryngoscope. 2017;128:248–56.

    Article  PubMed  Google Scholar 

  30. Smith JL. Whence pseudotumor cerebri? J Clin Neuroopthalmol. 1985;5:55–6.

    CAS  Google Scholar 

  31. Friedman D, Jacobson D. Diagnostic criteria for idiopathic intracranial hypertension. Neurology. 2002;59:1492–5.

    Article  PubMed  Google Scholar 

  32. Kupersmith M, 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;50:1094–8.

    Article  PubMed  CAS  Google Scholar 

  33. Johnson L, Krohel G, Madsen R, March G. The role of weight loss and acetazolamide in the treatment of idiopathic intracranial hypertension (pseudotumor cerebri). Ophthalmology. 1998;105:2313–7.

    Article  PubMed  CAS  Google Scholar 

  34. Handley J, Baruah B, Williams D, Horner M, Barry J, Stephens J. Bariatric surgery as a treatment for idiopathic intracranial hypertension: a systematic review. Surg Obes Relat Dis. 2015;11:1396–403.

    Article  PubMed  Google Scholar 

  35. • Ottridge R, Mollan S, Botfield H, et al. Randomised controlled trial of bariatric surgery versus a community weight loss programme for the sustained treatment of idiopathic intracranial hypertension: the idiopathic intracranial hypertension weight trial (IIH:WT) protocol. BMJ Open. 2017;7:e017426. Ongoing trial comparing bariatric surgery vs. weight loss program for treatment of primary PTCS.

    Article  PubMed  PubMed Central  Google Scholar 

  36. •• Smith S, Friedman D. The idiopathic intracranial hypertension treatment trial: a review of the outcomes. Headache J Head Face Pain. 2017;57:1303–10. Review summarizing the results of the IIHTT since 2014.

    Article  Google Scholar 

  37. Thurtell M, Wall M. Idiopathic intracranial hypertension (pseudotumor cerebri): recognition, treatment, and ongoing management. Curr Treat Options Neurol. 2012;15:1–12.

    Article  Google Scholar 

  38. Huna-Baron R, Kupersmith M. Idiopathic intracranial hypertension in pregnancy. J Neurol. 2002;249:1078–81.

    Article  PubMed  Google Scholar 

  39. Mukherjee N, Bhatti M. Update on the surgical management of idiopathic intracranial hypertension. Curr Neurol Neurosci Rep. 2014;14:438. https://doi.org/10.1007/s11910-014-0438-8.

    Article  PubMed  CAS  Google Scholar 

  40. Fonseca P, Rigamonti D, Miller N, Subramanian P. Visual outcomes of surgical intervention for pseudotumour cerebri: optic nerve sheath fenestration versus cerebrospinal fluid diversion. Br J Ophthalmol. 2014;98:1360–3.

    Article  PubMed  Google Scholar 

  41. deSouza R, Toma A, Watkins L. Medication overuse headache—an under-diagnosed problem in shunted idiopathic intracranial hypertension patients. Br J Neurosurg. 2014;29:30–4.

    Article  Google Scholar 

  42. Puffer R, Mustafa W, Lanzino G. Venous sinus stenting for idiopathic intracranial hypertension: a review of the literature. J NeuroInterv Surg. 2013;5:483–6.

    Article  PubMed  Google Scholar 

  43. Satti S, Leishangthem L, Chaudry M. Meta-analysis of CSF diversion procedures and dural venous sinus stenting in the setting of medically refractory idiopathic intracranial hypertension. Am J Neuroradiol. 2015;36:1899–904.

    Article  PubMed  CAS  Google Scholar 

  44. (2018) 7.1.1 Headache attributed to idiopathic intracranial hypertension (IIH) - ICHD-3 The International Classification of Headache Disorders 3rd edition. In: ICHD-3 The International Classification of Headache Disorders 3rd edition. https://www.ichd-3.org/7-headache-attributed-to-non-vascular-intracranial-disorder/7-1-headache-attributed-to-increased-cerebrospinal-fluid-pressure/7-1-1-headache-attributed-to-idiopathic-intracranial-hypertension-iih/. Accessed 2 Apr 2018.

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Authors and Affiliations

  1. Department of Neurology, Medstar Georgetown University Hospital, 3800 Reservoir Road NW, 7-PHC, Washington, DC, 20007, USA

    John Glenn Burkett & Jessica Ailani

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  1. John Glenn Burkett
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Correspondence to John Glenn Burkett.

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Conflict of Interest

John Glenn Burkett declares no potential conflicts of interest.

Jessica Ailani has received: grant support from Allergan and Theranica, and honoraria from Allergan (advisory board, speaking), Alder (advisory board, speaking), Amgen (advisory board, speaking), Avanir (speaking), Eli Lily and company (advisory board, speaking), Electrocore (advisory board, speaking), Promius (advisory board, speaking), and Teva (advisory board, speaking).

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This article does not contain any studies with human or animal subjects performed by any of the authors.

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Burkett, J.G., Ailani, J. An Up to Date Review of Pseudotumor Cerebri Syndrome. Curr Neurol Neurosci Rep 18, 33 (2018). https://doi.org/10.1007/s11910-018-0839-1

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