Skip to main content

Advertisement

Log in

A Review of Spontaneous Intracranial Hypotension

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

Abstract

Purpose of Review

Spontaneous intracranial hypotension (SIH) is an underdiagnosed phenomenon predominantly presenting with low cerebrospinal fluid (CSF) pressure and postural headache in setting of CSF leak. The goal of this paper is to provide updates on the pathology, diagnosis, and management of SIH. The utility of multiple imaging modalities and the use of epidural blood patches and fibrin glue polymers are explored.

Recent Findings

In regard to diagnosis, new non-invasive modalities in detection of SIH including transorbital ultrasound and serum biomarkers are found. In addition, increased efficacy of large volume and repeated placement of multiple epidural blood patches (EBP) are seen. In addition, the management of refractory SIH using fibrin glue polymers has proved efficacious in recent case series.

Summary

While the diagnosis may be challenging for clinicians, future research in SIH is leading to more rapid detection methods. Future studies may target optimal use of EBP in comparison to fibrin glue polymers, in addition to new developments in increased understanding of SIH physiology and phenotype.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

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

  1. Bier A. Experiments regarding the cocainization of the spinal cord. Surv Anesthesiol. 1962;6(3).

    Article  Google Scholar 

  2. Schaltenbrand G. Normal and pathological physiology of the cerebrospinal fluid circulation. Lancet. 1953;261(6765):805–8.

    Article  Google Scholar 

  3. Schievink WI, Morreale VM, Atkinson JLD, Meyer FB, Piepgras DG, Ebersold MJ. Surgical treatment of spontaneous spinal cerebrospinal fluid leaks. J Neurosurg. 1998;88:243–6.

    Article  CAS  PubMed  Google Scholar 

  4. Reinstein E, Pariani M, Bannykh S, Rimoin DL, Schievink WI. Connective tissue spectrum abnormalities associated with spontaneous cerebrospinal fluid leaks: a prospective study. Eur J Hum Genet. 2012;21(4):386–90.

    Article  PubMed  PubMed Central  Google Scholar 

  5. Schievink WI, Goseland A, Cunneen S. Bariatric surgery as a possible risk factor for spontaneous intracranial hypotension. Neurology. 2014;83(20):1819–22.

    Article  PubMed  Google Scholar 

  6. • He F-F, Li L, Liu M-J, Zhong T-D, Zhang Q-W, Fang X-M. Targeted epidural blood patch treatment for refractory spontaneous intracranial hypotension in China. J Neurol Surg B Skull Base. 2017;79(03):217–23 A large recent study of SIH patients that describes the spectrum of symptomology, comparison imaging modalities, and efficacy of targeted patches.

    Article  PubMed  Google Scholar 

  7. Zada G, Solomon TC, Giannotta SL. A review of ocular manifestations in intracranial hypotension. Neurosurg Focus. 2007;23.

    Article  PubMed  Google Scholar 

  8. Cheshire WP, Wharen RE. Trigeminal neuralgia in a patient with spontaneous intracranial hypotension. Headache. 2009;49(5):770–3.

    Article  PubMed  Google Scholar 

  9. Russo A, Tessitore A, Cirillo M, Giordano A, Micco RD, Bussone G, et al. A transient third cranial nerve palsy as presenting sign of spontaneous intracranial hypotension. J Headache Pain. 2011;12(4):493–6.

    Article  PubMed  PubMed Central  Google Scholar 

  10. Pakiam AS-I, Lee C, Lang AE. Intracranial hypotension with parkinsonism, ataxia, and bulbar weakness. Arch Neurol. 1999;56(7):869.

    Article  CAS  PubMed  Google Scholar 

  11. Lagrand TJ, Beukers R. Sagging brain causing postural loss of consciousness: a case of severe spontaneous intracranial hypotension. Pract Neurol. 2015;15(6):471–3.

    Article  PubMed  Google Scholar 

  12. Fishman RA, Dillon WP, Foster N, Hong M, Shah G, Turner R, et al. Spontaneous intracranial hypotension causing reversible frontotemporal dementia. Neurology. 2002;59(5):787.

    Article  PubMed  Google Scholar 

  13. Mokri B, Atkinson JLD, Piepgras DG. Absent headache despite CSF volume depletion (intracranial hypotension). Neurology. 2000;55(11):1722–4.

    Article  CAS  PubMed  Google Scholar 

  14. Mokri B, Aksamit A, Atkinson J. Paradoxical postural headaches in cerebrospinal fluid leaks. Cephalalgia. 2004;24(10):883–7.

    Article  CAS  PubMed  Google Scholar 

  15. • Qureshi AI, Kherani D, Waqas MA, Qureshi MH, Raja FM, Wallery SS. Effect of epidural blood injection on upright posture intolerance in patients with headaches due to intracranial hypotension: a prospective study. Brain Behav. 2018;8(7) A study looking at the timing of postural intolerance in SIH.

    Article  PubMed  PubMed Central  Google Scholar 

  16. • Fujii N, Fujii H, Fujita A, Kim Y, Sugimoto H. Spontaneous intracranial hypotension complicated by cerebral venous thrombosis. Radiol Case Rep. 2018;13(4):834–8 A case report that describes a rare complication of SIH.

    Article  PubMed  PubMed Central  Google Scholar 

  17. Mokri B, Hunter SF, Atkinson J, Piepgras DG. Orthostatic headaches caused by CSF leak but with normal CSF pressures. Neurology. 1998;51(3):786–90.

    Article  CAS  PubMed  Google Scholar 

  18. Schievink WI, Gordon OK, Tourje J. Connective tissue disorders with spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension: a prospective study. Neurosurgery. 2004;54(1):65–71.

    Article  PubMed  Google Scholar 

  19. Schievink WI, Jacques L. Recurrent spontaneous spinal cerebrospinal fluid leak associated with “nude nerve root” syndrome: case report. Neurosurgery. 2003;53(5):1216–9.

    Article  PubMed  Google Scholar 

  20. Beck J, Ulrich CT, Fung C, Fichtner J, Seidel K, Fiechter M, et al. Diskogenic microspurs as a major cause of intractable spontaneous intracranial hypotension. Neurology. 2016;87(12):1220–6.

    Article  PubMed  Google Scholar 

  21. Schievink WI, Meyer FB, Atkinson JLD, Mokri B. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. J Neurosurg. 1996;84:598–605.

    Article  CAS  PubMed  Google Scholar 

  22. Schievink WI, Reimer R, Folger WN. Surgical treatment of spontaneous intracranial hypotension associated with a spinal arachnoid diverticulum. J Neurosurg. 1994;80:736–9.

    Article  CAS  PubMed  Google Scholar 

  23. Mokri B. The Monro-Kellie hypothesis: applications in CSF volume depletion. Neurology. 2001;56(12):1746–8.

    Article  CAS  PubMed  Google Scholar 

  24. • Headache Classification Committee of the International Headache Society (IHS). The International Classification of Headache Disorders, 3rd edition. Cephalalgia. 2018;38(1):1–211 New criteria of diagnosis of SIH.

    Article  Google Scholar 

  25. Schievink WI. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. Jama. 2006;295(19):2286.

    Article  CAS  PubMed  Google Scholar 

  26. Mokri B, Low PA. Orthostatic headaches without CSF leak in postural tachycardia syndrome. Neurology. 2003;61(7):980–2.

    Article  PubMed  Google Scholar 

  27. Mokri B, Piepgras DG, Miller GM. Syndrome of orthostatic headaches and diffuse pachymeningeal gadolinium enhancement. Mayo Clin Proc. 1997;72(5):400–13.

    Article  CAS  PubMed  Google Scholar 

  28. Kranz PG, Malinzak MD, Amrhein TJ, Gray L. Update on the diagnosis and treatment of spontaneous intracranial hypotension. Curr Pain Headache Rep. 2017;21(8).

  29. Kranz PG, Amrhein TJ, Choudhury KR, Tanpitukpongse TP, Gray L. Time-dependent changes in dural enhancement associated with spontaneous intracranial hypotension. Am J Roentgenol. 2016;207(6):1283–7.

    Article  Google Scholar 

  30. Alvarez-Linera J, Escribano J, Benito-Leon J, Porta-Etessam J, Rovira A. Pituitary enlargement in patients with intracranial hypotension syndrome. Neurology. 2000;55(12):1895–7.

    Article  CAS  PubMed  Google Scholar 

  31. Kranz PG, Tanpitukpongse TP, Choudhury KR, Amrhein TJ, Gray L. Imaging signs in spontaneous intracranial hypotension: prevalence and relationship to CSF pressure. AJNR. 2016;37(7):1374–8.

    Article  CAS  PubMed  Google Scholar 

  32. Watanabe A, Horikoshi T, Uchida M, Koizumi H, Yagishita T, Kinouchi H. Diagnostic value of spinal MR imaging in spontaneous intracranial hypotension syndrome. AJNR. 2008;30(1):147–51.

    Article  PubMed  Google Scholar 

  33. Luetmer PH, Mokri B. Dynamic CT myelography: a technique for localizing high-flow spinal cerebrospinal fluid leaks. AJNR Am J Neuroradiol. 2003;24:1711–4.

    PubMed  Google Scholar 

  34. Hoxworth J, Patel A, Bosch E, Nelson K. Localization of a rapid CSF leak with digital subtraction myelography. Am J Neuroradiol. 2008;30(3):516–9.

    Article  PubMed  Google Scholar 

  35. Akbar J, Luetmer P, Schwartz K, Hunt C, Diehn F, Eckel L. The role of MR myelography with intrathecal gadolinium in localization of spinal CSF leaks in patients with spontaneous intracranial hypotension. Am J Neuroradiol. 2011;33(3):535–40.

    Article  PubMed  Google Scholar 

  36. Albayram S, Kilic F, Ozer H, Baghaki S, Kocer N, Islak C. Gadolinium-enhanced MR cisternography to evaluate dural leaks in intracranial hypotension syndrome. Am J Neuroradiol. 2007;29(1):116–21.

    Article  PubMed  Google Scholar 

  37. • Young SJ, Quisling RG, Bidari S, Sanghvi TS. An objective study of anatomic shifts in intracranial hypotension using four anatomic planes. Radiol Res Pract. 2018;2018:1–9 This study aims to create quantifiable measurements on cranial MRI for diagnosis and stratification of SIH patients.

    Article  Google Scholar 

  38. Kranz PG, Tanpitukpongse TP, Choudhury KR, Amrhein TJ, Gray L. How common is normal cerebrospinal fluid pressure in spontaneous intracranial hypotension? Cephalalgia. 2016;36(13):1209–17.

    Article  PubMed  Google Scholar 

  39. Fichtner J, Ulrich CT, Fung C, Knüppel C, Veitweber M, Jilch A, et al. Management of spontaneous intracranial hypotension – Transorbital ultrasound as discriminator. J Neurol Neurosurg Psychiatry. 2015;87(6):650–5.

    Article  PubMed  PubMed Central  Google Scholar 

  40. • Murakami Y, Takahashi K, Hoshi K, Ito H, Kanno M, Saito K, et al. Spontaneous intracranial hypotension is diagnosed by a combination of lipocalin-type prostaglandin D synthase and brain-type transferrin in cerebrospinal fluid. Biochim Biophys Acta Gen Subj. 2018;1862(8):1835–42 This study aims to determine potential serum biomarkers for SIH.

    Article  CAS  PubMed  Google Scholar 

  41. Wu J-W, Hseu S-S, Fuh J-L, Lirng J-F, Wang Y-F, Chen W-T, et al. Factors predicting response to the first epidural blood patch in spontaneous intracranial hypotension. Brain. 2017;140(2):344–52.

    Article  PubMed  Google Scholar 

  42. Karm M-H, Choi J-H, Kim D, Park JY, Yun HJ, Suh JH. Predictors of the treatment response of spontaneous intracranial hypotension to an epidural blood patch. Medicine. 2016;95(18):e3578.

    Article  PubMed  PubMed Central  Google Scholar 

  43. • Lee JY, Lee MJ, Park HJ, Park JH, Jeong HJ, Oh MS, et al. Clinical effect of the proximity of epidural blood patch injection to the leakage site in spontaneous intracranial hypotension. Br J Neurosurg. 2018;32(6):671–3 A recent finding that no difference between the location of CSF leak and the placement of EBP in response rate.

    Article  PubMed  Google Scholar 

  44. Özütemiz C, Köksel YK, Huang H, Rubin N, Rykken JB. The efficacy of fluoroscopy-guided epidural blood patch in the treatment of spontaneous and iatrogenic cerebrospinal fluid leakage. Eur Radiol. 2018.

  45. • Ohtonari T, Ota S, Himeno T, Nishihara N, Sato M, Tanaka A. Excellent outcomes of large-volume epidural blood patch using an intravenous catheter in 15 consecutive cases with cerebrospinal fluid leak. World Neurosurg. 2018;118. – A recent study finding the benefit with higher volume epidural blood patches.:e276–82.

    Article  PubMed  Google Scholar 

  46. • Ahn C, Lee E, Lee JW, Chee CG, Kang Y, Kang HS. Two-site blind epidural blood patch versus targeted epidural blood patch in spontaneous intracranial hypotension. J Clin Neurosci. 2018; A recent study on the utility of localization of CSF leak and placement of epidural blood patch.

  47. Tonnelet R, Colnat-Coulbois S, Mione G, Richard S, Bouaziz H, Audibert G, et al. Successful treatment of spontaneous intracranial hypotension by plugging the cerebrospinal fluid leak with percutaneous cyanoacrylate injection: a report of 2 cases. World Neurosurg. 2016;91:390–8.

    Article  PubMed  Google Scholar 

  48. Cohen-Gadol AA, Mokri B, Piepgras DG, Meyer FB, Atkinson JL. Surgical anatomy of dural defects in spontaneous spinal cerebrospinal fluid leaks. Oper Neurosurg. 2006;58:ONS–238-ONS-245.

    Article  Google Scholar 

  49. Mokri B. Intracranial hypertension after treatment of spontaneous cerebrospinal fluid leaks. Mayo Clin Proc. 2002;77(11):1241–6.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Parth Upadhyaya.

Ethics declarations

Conflict of Interest

Jessica Ailani reports personal fees from Allergan, Alder, Amgen, Avanir, Eli Lilly, Biohaven, Teva, Impel, Promius, Electrocore, AlphaSites Consulting, Miller Medical Communications, Aptus Health, and Current Pain and Headache Reports, outside the submitted work. Parth Upadhyaya declares no potential conflicts of interest.

Human and Animal Rights and Informed Consent

All reported studies/experiments with human or animal subjects performed by the authors have been previously published and complied with all applicable ethical standards (including the Helsinki declaration and its amendments, institutional/national research committee standards, and international/national/institutional guidelines).

Additional information

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

This article is part of the Topical Collection on Headache

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Upadhyaya, P., Ailani, J. A Review of Spontaneous Intracranial Hypotension. Curr Neurol Neurosci Rep 19, 22 (2019). https://doi.org/10.1007/s11910-019-0938-7

Download citation

  • Published:

  • DOI: https://doi.org/10.1007/s11910-019-0938-7

Keywords

Navigation