A Review of Spontaneous Intracranial Hypotension

  • Parth UpadhyayaEmail author
  • Jessica Ailani
Headache (R. Halker Singh, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Headache


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.


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.


SIH, spontaneous intracranial hypotension CSF leak Epidural blood patch Postural headache Fibrin glue 


Compliance with Ethical Standards

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).


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

  1. 1.
    Bier A. Experiments regarding the cocainization of the spinal cord. Surv Anesthesiol. 1962;6(3).CrossRefGoogle Scholar
  2. 2.
    Schaltenbrand G. Normal and pathological physiology of the cerebrospinal fluid circulation. Lancet. 1953;261(6765):805–8.CrossRefGoogle Scholar
  3. 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.CrossRefPubMedGoogle Scholar
  4. 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.CrossRefPubMedPubMedCentralGoogle Scholar
  5. 5.
    Schievink WI, Goseland A, Cunneen S. Bariatric surgery as a possible risk factor for spontaneous intracranial hypotension. Neurology. 2014;83(20):1819–22.CrossRefPubMedGoogle Scholar
  6. 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. CrossRefPubMedGoogle Scholar
  7. 7.
    Zada G, Solomon TC, Giannotta SL. A review of ocular manifestations in intracranial hypotension. Neurosurg Focus. 2007;23.CrossRefPubMedGoogle Scholar
  8. 8.
    Cheshire WP, Wharen RE. Trigeminal neuralgia in a patient with spontaneous intracranial hypotension. Headache. 2009;49(5):770–3.CrossRefPubMedGoogle Scholar
  9. 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.CrossRefPubMedPubMedCentralGoogle Scholar
  10. 10.
    Pakiam AS-I, Lee C, Lang AE. Intracranial hypotension with parkinsonism, ataxia, and bulbar weakness. Arch Neurol. 1999;56(7):869.CrossRefPubMedGoogle Scholar
  11. 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.CrossRefPubMedGoogle Scholar
  12. 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.CrossRefPubMedGoogle Scholar
  13. 13.
    Mokri B, Atkinson JLD, Piepgras DG. Absent headache despite CSF volume depletion (intracranial hypotension). Neurology. 2000;55(11):1722–4.CrossRefPubMedGoogle Scholar
  14. 14.
    Mokri B, Aksamit A, Atkinson J. Paradoxical postural headaches in cerebrospinal fluid leaks. Cephalalgia. 2004;24(10):883–7.CrossRefPubMedGoogle Scholar
  15. 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. CrossRefPubMedPubMedCentralGoogle Scholar
  16. 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. CrossRefPubMedPubMedCentralGoogle Scholar
  17. 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.CrossRefPubMedGoogle Scholar
  18. 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.CrossRefPubMedGoogle Scholar
  19. 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.CrossRefPubMedGoogle Scholar
  20. 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.CrossRefPubMedGoogle Scholar
  21. 21.
    Schievink WI, Meyer FB, Atkinson JLD, Mokri B. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. J Neurosurg. 1996;84:598–605.CrossRefPubMedGoogle Scholar
  22. 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.CrossRefPubMedGoogle Scholar
  23. 23.
    Mokri B. The Monro-Kellie hypothesis: applications in CSF volume depletion. Neurology. 2001;56(12):1746–8.CrossRefPubMedGoogle Scholar
  24. 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. CrossRefGoogle Scholar
  25. 25.
    Schievink WI. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. Jama. 2006;295(19):2286.CrossRefPubMedGoogle Scholar
  26. 26.
    Mokri B, Low PA. Orthostatic headaches without CSF leak in postural tachycardia syndrome. Neurology. 2003;61(7):980–2.CrossRefPubMedGoogle Scholar
  27. 27.
    Mokri B, Piepgras DG, Miller GM. Syndrome of orthostatic headaches and diffuse pachymeningeal gadolinium enhancement. Mayo Clin Proc. 1997;72(5):400–13.CrossRefPubMedGoogle Scholar
  28. 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).Google Scholar
  29. 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.CrossRefGoogle Scholar
  30. 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.CrossRefPubMedGoogle Scholar
  31. 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.CrossRefPubMedGoogle Scholar
  32. 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.CrossRefPubMedGoogle Scholar
  33. 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.PubMedGoogle Scholar
  34. 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.CrossRefPubMedGoogle Scholar
  35. 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.CrossRefPubMedGoogle Scholar
  36. 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.CrossRefPubMedGoogle Scholar
  37. 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. CrossRefGoogle Scholar
  38. 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.CrossRefPubMedGoogle Scholar
  39. 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.CrossRefPubMedPubMedCentralGoogle Scholar
  40. 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. CrossRefPubMedGoogle Scholar
  41. 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.CrossRefPubMedGoogle Scholar
  42. 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.CrossRefPubMedPubMedCentralGoogle Scholar
  43. 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. CrossRefPubMedGoogle Scholar
  44. 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.Google Scholar
  45. 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.CrossRefPubMedGoogle Scholar
  46. 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. Google Scholar
  47. 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.CrossRefPubMedGoogle Scholar
  48. 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.CrossRefGoogle Scholar
  49. 49.
    Mokri B. Intracranial hypertension after treatment of spontaneous cerebrospinal fluid leaks. Mayo Clin Proc. 2002;77(11):1241–6.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of NeurologyMedstar Georgetown University HospitalWashingtonUSA
  2. 2.Department of Neurology, Medstar Georgetown Headache CenterMedstar Georgetown University HospitalWashingtonUSA

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