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Update on the Diagnosis and Treatment of Spontaneous Intracranial Hypotension

  • Peter G. KranzEmail author
  • Michael D. Malinzak
  • Timothy J. Amrhein
  • Linda Gray
Migraine and Beyond (R Cowan, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Migraine and Beyond

Abstract

Purpose of Review

The purpose of this study is to provide an update on recent developments in the understanding, diagnosis, and treatment of spontaneous intracranial hypotension (SIH).

Recent Findings

SIH is an important cause of headaches caused by spinal cerebrospinal fluid (CSF) leaks, with an increasingly broad spectrum of clinical presentations and diagnostic findings. A simple conception of the condition as being defined by the presence of low CSF pressure is no longer sufficient or accurate. A number of etiologies for spinal CSF leaks have been identified, including the recent discovery of CSF-venous fistulas, and these various etiologies may require different diagnostic and therapeutic pathways in order to affect a cure.

Summary

Familiarity with the spectrum of presentations and causes of SIH is critical to accurate and timely diagnosis and management. Challenges exist in both diagnosis and treatment, and require understanding of the underlying pathogenesis of the condition in order to appropriately select testing and treatment. Prospective studies are needed going forward in order to inform workup and guide treatment decisions.

Keywords

Spontaneous intracranial hypotension CSF leak Epidural blood patch Cerebrospinal fluid CSF-venous fistula 

Notes

Compliance with Ethical Standards

Conflict of Interest

Peter G. Kranz, Michael D. Malinzak, Timothy J. Amrhein, and Linda Gray each declare no potential conflicts of interest.

Human and Animal Rights and Informed Consent

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

References

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

  1. 1.
    Mokri B. Headaches caused by decreased intracranial pressure: diagnosis and management. Curr Opin Neurol. 2003;16(3):319–26. doi: 10.1097/01.wco.0000073933.19076.c0.CrossRefPubMedGoogle Scholar
  2. 2.
    • Kranz PG, Tanpitukpongse TP, Choudhury KR, Amrhein TJ, Gray L. How common is normal cerebrospinal fluid pressure in spontaneous intracranial hypotension? Cephalalgia. 2015; doi: 10.1177/0333102415623071. Analysis of patients with SIH finding that normal CSF opening pressure is more common than finding a pressure <6 cm H 2 O.
  3. 3.
    Luetmer PH, Schwartz KM, Eckel LJ, Hunt CH, Carter RE, Diehn FE. When should I do dynamic CT myelography? Predicting fast spinal CSF leaks in patients with spontaneous intracranial hypotension. AJNR Am J Neuroradiol. 2012;33(4):690–4. doi: 10.3174/ajnr.A2849.CrossRefPubMedGoogle Scholar
  4. 4.
    Miyazawa K, Shiga Y, Hasegawa T, Endoh M, Okita N, Higano S, et al. CSF hypovolemia vs intracranial hypotension in "spontaneous intracranial hypotension syndrome". Neurology. 2003;60(6):941–7. doi: 10.1212/01.wnl.0000049933.51044.81.CrossRefPubMedGoogle Scholar
  5. 5.
    • Mokri B. Spontaneous cerebrospinal fluid leaks: from intracranial hypotension to cerebrospinal fluid hypovolemia—evolution of a concept. Mayo Clin Proc. 1999;74(11):1113–23. doi: 10.4065/74.11.1113. Critical discussion of the role of CSF pressure versus CSF volume in SIH. CrossRefPubMedGoogle Scholar
  6. 6.
    Schievink WI, Morreale VM, Atkinson JL, Meyer FB, Piepgras DG, Ebersold MJ. Surgical treatment of spontaneous spinal cerebrospinal fluid leaks. J Neurosurg. 1998;88(2):243–6. doi: 10.3171/jns.1998.88.2.0243.CrossRefPubMedGoogle Scholar
  7. 7.
    Schievink WI, Meyer FB, Atkinson JL, Mokri B. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. J Neurosurg. 1996;84(4):598–605. doi: 10.3171/jns.1996.84.4.0598.CrossRefPubMedGoogle Scholar
  8. 8.
    Kranz PG, Stinnett SS, Huang KT, Gray L. Spinal meningeal diverticula in spontaneous intracranial hypotension: analysis of prevalence and myelographic appearance. AJNR Am J Neuroradiol. 2013;34(6):1284–9. doi: 10.3174/ajnr.A3359.CrossRefPubMedGoogle Scholar
  9. 9.
    Schievink WI. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. JAMA. 2006;295(19):2286–96. doi: 10.1001/jama.295.19.2286.CrossRefPubMedGoogle Scholar
  10. 10.
    Chen CH, Chen JH, Chen HC, Chai JW, Chen PL, Chen CC. Patterns of cerebrospinal fluid (CSF) distribution in patients with spontaneous intracranial hypotension: assessed with magnetic resonance myelography. J Chin Med Assoc. 2017;80(2):109–16. doi: 10.1016/j.jcma.2016.02.013.CrossRefPubMedGoogle Scholar
  11. 11.
    Mokri B, Maher CO, Sencakova D. Spontaneous CSF leaks: underlying disorder of connective tissue. Neurology. 2002;58(5):814–6. doi: 10.1212/wnl.58.5.814.CrossRefPubMedGoogle Scholar
  12. 12.
    Mokri B. Spontaneous low pressure, low CSF volume headaches: spontaneous CSF leaks. Headache. 2013;53(7):1034–53. doi: 10.1111/head.12149.CrossRefPubMedGoogle Scholar
  13. 13.
    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. doi: 10.1212/WNL.0000000000003122.CrossRefPubMedGoogle Scholar
  14. 14.
    •• Kranz PG, Luetmer PH, Diehn FE, Amrhein TJ, Tanpitukpongse TP, Gray L. Myelographic techniques for the detection of spinal CSF leaks in spontaneous intracranial hypotension. AJR Am J Roentgenol. 2016;206(1):8–19. doi: 10.2214/AJR.15.14884. Review of causes of spinal CSF leaks and the imaging techniques used to identify them. CrossRefPubMedGoogle Scholar
  15. 15.
    Yoshida H, Takai K, Taniguchi M. Leakage detection on CT myelography for targeted epidural blood patch in spontaneous cerebrospinal fluid leaks: calcified or ossified spinal lesions ventral to the thecal sac. J Neurosurg Spine. 2014;21(3):432–41. doi: 10.3171/2014.5.SPINE13549.CrossRefPubMedGoogle Scholar
  16. 16.
    Thielen KR, Sillery JC, Morris JM, Hoxworth JM, Diehn FE, Wald JT, et al. Ultrafast dynamic computed tomography myelography for the precise identification of high-flow cerebrospinal fluid leaks caused by spiculated spinal osteophytes. J Neurosurg Spine. 2015;22(3):324–31. doi: 10.3171/2014.10.SPINE14209.CrossRefPubMedGoogle Scholar
  17. 17.
    Gille O, Soderlund C, Razafimahandri HJ, Mangione P, Vital JM. Analysis of hard thoracic herniated discs: review of 18 cases operated by thoracoscopy. Eur Spine J. 2006;15(5):537–42. doi: 10.1007/s00586-005-1014-3.CrossRefPubMedPubMedCentralGoogle Scholar
  18. 18.
    • Schievink WI, Moser FG, Maya MM, Prasad RS. Digital subtraction myelography for the identification of spontaneous spinal CSF-venous fistulas. J Neurosurg Spine. 2016;24(6):960–4. doi: 10.3171/2015.10.SPINE15855. Discussion of the role of digital subtraction myelography in identfying CSF-venous fistulas CrossRefPubMedGoogle Scholar
  19. 19.
    Kranz PG, Amrhein TJ, Schievink WI, Karikari IO, Gray L. The “Hyperdense Paraspinal vein” sign: a marker of CSF-venous fistula. AJNR Am J Neuroradiol. 2016;37(7):1379–81. doi: 10.3174/ajnr.A4682.CrossRefPubMedGoogle Scholar
  20. 20.
    •• Schievink WI, Moser FG, Maya MM. CSF-venous fistula in spontaneous intracranial hypotension. Neurology. 2014;83(5):472–3. doi: 10.1212/WNL.0000000000000639. Initial description of CSF-venous fistulas as a cause of SIH. CrossRefPubMedGoogle Scholar
  21. 21.
    Magnaes B. Body position and cerebrospinal fluid pressure. Part 1: clinical studies on the effect of rapid postural changes. J Neurosurg. 1976;44(6):687–97. doi: 10.3171/jns.1976.44.6.0687.CrossRefPubMedGoogle Scholar
  22. 22.
    Schievink WI, Schwartz MS, Maya MM, Moser FG, Rozen TD. Lack of causal association between spontaneous intracranial hypotension and cranial cerebrospinal fluid leaks. J Neurosurg. 2012;116(4):749–54. doi: 10.3171/2011.12.JNS111474.CrossRefPubMedGoogle Scholar
  23. 23.
    Schievink WI, Maya MM, Moser F, Tourje J, Torbati S. Frequency of spontaneous intracranial hypotension in the emergency department. J Headache Pain. 2007;8(6):325–8. doi: 10.1007/s10194-007-0421-8.CrossRefPubMedPubMedCentralGoogle Scholar
  24. 24.
    Schievink WI. Misdiagnosis of spontaneous intracranial hypotension. Arch Neurol. 2003;60(12):1713–8. doi: 10.1001/archneur.60.12.1713.CrossRefPubMedGoogle Scholar
  25. 25.
    Kranz PG, Amrhein TJ, Choudhury KR, Tanpitukpongse TP, Gray L. Time-dependent changes in dural enhancement associated with spontaneous intracranial hypotension. AJR Am J Roentgenol. 2016;207(6):1283–7. doi: 10.2214/AJR.16.16381.CrossRefPubMedGoogle Scholar
  26. 26.
    Kranz PG, Tanpitukpongse TP, Choudhury KR, Amrhein TJ, Gray L. Imaging signs in spontaneous intracranial hypotension: prevalence and relationship to CSF pressure. AJNR Am J Neuroradiol. 2016;37(7):1374–8. doi: 10.3174/ajnr.A4689.CrossRefPubMedGoogle Scholar
  27. 27.
    Tanaka Y, Tosaka M, Fujimaki H, Honda F, Yoshimoto Y. Sex- and age-related differences in the clinical and neuroimaging characteristics of patients with spontaneous intracranial hypotension: a records review. Headache. 2016;56(8):1310–6. doi: 10.1111/head.12887.CrossRefPubMedGoogle Scholar
  28. 28.
    Capizzano AA, Lai L, Kim J, Rizzo M, Gray L, Smoot MK, et al. Atypical presentations of intracranial hypotension: comparison with classic spontaneous intracranial hypotension. AJNR Am J Neuroradiol. 2016;37(7):1256–61. doi: 10.3174/ajnr.A4706.CrossRefPubMedGoogle Scholar
  29. 29.
    Mokri B, Low PA. Orthostatic headaches without CSF leak in postural tachycardia syndrome. Neurology. 2003;61(7):980–2. doi: 10.1212/01.wnl.0000085868.37963.7d.CrossRefPubMedGoogle Scholar
  30. 30.
    • Headache Classification Committee of the International Headache S. The international classification of headache disorders, 3rd edition (beta version). Cephalalgia. 2013;33(9):629–808. doi: 10.1177/0333102413485658. Most widely accepted diagnostic criteria in SIH. CrossRefGoogle Scholar
  31. 31.
    Farb RI, Forghani R, Lee SK, Mikulis DJ, Agid R. The venous distension sign: a diagnostic sign of intracranial hypotension at MR imaging of the brain. AJNR Am J Neuroradiol. 2007;28(8):1489–93. doi: 10.3174/ajnr.A0621.CrossRefPubMedGoogle Scholar
  32. 32.
    Chazen JL, Talbott JF, Lantos JE, Dillon WP. MR myelography for identification of spinal CSF leak in spontaneous intracranial hypotension. AJNR Am J Neuroradiol. 2014;35(10):2007–12. doi: 10.3174/ajnr.A3975.CrossRefPubMedGoogle Scholar
  33. 33.
    Wendl CM, Schambach F, Zimmer C, Forschler A. CT myelography for the planning and guidance of targeted epidural blood patches in patients with persistent spinal CSF leakage. AJNR Am J Neuroradiol. 2012;33(3):541–4. doi: 10.3174/ajnr.A2808.CrossRefPubMedGoogle Scholar
  34. 34.
    Yao LL, Hu XY. Factors affecting cerebrospinal fluid opening pressure in patients with spontaneous intracranial hypotension. J Zhejiang Univ Sci B. 2017;18(7):577–85. doi: 10.1631/jzus.B1600343.
  35. 35.
    Verdoorn JT, Luetmer PH, Carr CM, Lane JI, Lehman VT, Morris JM, et al. Predicting high-flow spinal CSF leaks in spontaneous intracranial hypotension using a spinal MRI-based algorithm: have repeat CT myelograms been reduced? AJNR Am J Neuroradiol. 2016;37(1):185–8. doi: 10.3174/ajnr.A4465.CrossRefPubMedGoogle Scholar
  36. 36.
    Griauzde J, Gemmete JJ, Pandey AS, Chaudhary N. Intrathecal preservative-free normal saline challenge magnetic resonance myelography for the identification of cerebrospinal fluid leaks in spontaneous intracranial hypotension. J Neurosurg. 2015;123(3):732–6. doi: 10.3171/2014.12.JNS142057.CrossRefPubMedGoogle Scholar
  37. 37.
    Rando TA, Fishman RA. Spontaneous intracranial hypotension: report of two cases and review of the literature. Neurology. 1992;42(3 Pt 1):481–7.CrossRefPubMedGoogle Scholar
  38. 38.
    Agarwal V, Sreedher G, Rothfus WE. Targeted CT-guided epidural blood patch for treatment of spontaneous intracranial hypotension due to calcified intradural thoracic disc herniation. Interv Neuroradiol. 2013;19(1):121–6. doi: 10.1177/159101991301900118.CrossRefPubMedPubMedCentralGoogle Scholar
  39. 39.
    Chung SJ, Lee JH, Im JH, Lee MC. Short- and long-term outcomes of spontaneous CSF hypovolemia. Eur Neurol. 2005;54(2):63–7. doi: 10.1159/000087714.CrossRefPubMedGoogle Scholar
  40. 40.
    Kong DS, Park K, Nam DH, Lee JI, Kim JS, Eoh W, et al. Clinical features and long-term results of spontaneous intracranial hypotension. Neurosurgery. 2005;57(1):91–6. discussion -6CrossRefPubMedGoogle Scholar
  41. 41.
    Mokri B. Spontaneous CSF leaks: low CSF volume syndromes. Neurol Clin. 2014;32(2):397–422. doi: 10.1016/j.ncl.2013.11.002.CrossRefPubMedGoogle Scholar
  42. 42.
    Sencakova D, Mokri B, McClelland RL. The efficacy of epidural blood patch in spontaneous CSF leaks. Neurology. 2001;57(10):1921–3. doi: 10.1212/wnl.57.10.1921.CrossRefPubMedGoogle Scholar
  43. 43.
    Berroir S, Loisel B, Ducros A, Boukobza M, Tzourio C, Valade D, et al. Early epidural blood patch in spontaneous intracranial hypotension. Neurology. 2004;63(10):1950–1. doi: 10.1212/01.wnl.0000144339.34733.e9.CrossRefPubMedGoogle Scholar
  44. 44.
    Cho KI, Moon HS, Jeon HJ, Park K, Kong DS. Spontaneous intracranial hypotension: efficacy of radiologic targeting vs blind blood patch. Neurology. 2011;76(13):1139–44. doi: 10.1212/WNL.0b013e318212ab43.CrossRefPubMedGoogle Scholar
  45. 45.
    Schievink WI, Deline CR. Headache secondary to intracranial hypotension. Curr Pain Headache Rep. 2014;18(11):457. doi: 10.1007/s11916-014-0457-9.CrossRefPubMedGoogle Scholar
  46. 46.
    Schievink WI. A novel technique for treatment of intractable spontaneous intracranial hypotension: lumbar dural reduction surgery. Headache. 2009;49(7):1047–51. doi: 10.1111/j.1526-4610.2009.01450.x.CrossRefPubMedGoogle Scholar
  47. 47.
    • Kranz PG, Amrhein TJ, Gray L. Rebound intracranial hypertension: a complication of epidural blood patching for intracranial hypotension. AJNR Am J Neuroradiol. 2014;35(6):1237–40. doi: 10.3174/ajnr.A3841. Review of Rebound intracranial hypertension after blood patching, its presentation, and treatment. CrossRefPubMedGoogle Scholar
  48. 48.
    Karm MH, Choi JH, Kim D, Park JY, Yun HJ, Suh JH. Predictors of the treatment response of spontaneous intracranial hypotension to an epidural blood patch. Medicine (Baltimore). 2016;95(18):e3578. doi: 10.1097/MD.0000000000003578.CrossRefGoogle Scholar
  49. 49.
    Choi H, Lee MJ, Choi HA, Cha J, Chung CS. Intracranial structural alteration predicts treatment outcome in patients with spontaneous intracranial hypotension. Cephalalgia. 2017:333102417690106; doi: 10.1177/0333102417690106.
  50. 50.
    Wu JW, Hseu SS, Fuh JL, Lirng JF, Wang YF, Chen WT, et al. Factors predicting response to the first epidural blood patch in spontaneous intracranial hypotension. Brain. 2017;140(Pt 2):344–52. doi: 10.1093/brain/aww328.CrossRefPubMedGoogle Scholar
  51. 51.
    Schievink WI, Maya MM, Louy C. Cranial MRI predicts outcome of spontaneous intracranial hypotension. Neurology. 2005;64(7):1282–4. doi: 10.1212/01.WNL.0000156906.84165.C0.CrossRefPubMedGoogle Scholar
  52. 52.
    Amrhein TJ, Befera NT, Gray L, Kranz PG. CT fluoroscopy-guided blood patching of ventral CSF leaks by direct needle placement in the ventral epidural space using a transforaminal approach. AJNR Am J Neuroradiol. 2016; doi: 10.3174/ajnr.A4842.
  53. 53.
    Kranz P, Gray L, Amrhein A. CSF-venous fistulas in spontaneous intracranial hypotension: imaging characteristics on dynamic and CT myelography. AJR Am J Roentgenol. [In Press]Google Scholar
  54. 54.
    Takahashi K, Mima T, Akiba Y. Chronic subdural hematoma associated with spontaneous intracranial hypotension: therapeutic strategies and outcomes of 55 cases. Neurol Med Chir (Tokyo). 2016;56(2):69–76. doi: 10.2176/nmc.oa.2015-0032.CrossRefGoogle Scholar
  55. 55.
    Wan Y, Xie J, Xie D, Xue Z, Wang Y, Yang S. Clinical characteristics of 15 cases of chronic subdural hematomas due to spontaneous intracranial hypotension with spinal cerebrospinal fluid leak. Acta Neurol Belg. 2016;116(4):509–12. doi: 10.1007/s13760-016-0597-2.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2017

Authors and Affiliations

  • Peter G. Kranz
    • 1
    Email author
  • Michael D. Malinzak
    • 1
  • Timothy J. Amrhein
    • 1
  • Linda Gray
    • 1
  1. 1.Department of RadiologyDuke University Medical CenterDurhamUSA

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