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Spontaneous intracranial hypotension: review and expert opinion

Abstract

Spontaneous intracranial hypotension (SIH) results from spinal cerebrospinal fluid (CSF) leaking. An underlying connective tissue disorder that predisposes to weakness of the dura is implicated in spontaneous spinal CSF leaks. During the last decades, a much larger number of spontaneous cases are identified and a far broader clinical SIH spectrum is recognized. Orthostatic headache is the main presentation symptom of SIH; some patients also have other manifestations, mainly cochlear–vestibular signs and symptoms. Differential diagnosis with other syndromes presenting with orthostatic headache is crucial. Brain CT, brain MR, spine MRI, and MRI myelography are the imaging modalities of first choice for SIH diagnosis. Invasive imaging techniques, such as myelography, CT myelography, and radioisotopic cisternography, are progressively being abandoned. No randomized clinical trials have assessed the treatment of SIH. In a minority of cases, SIH resolved spontaneously or with only conservative treatment. If orthostatic headache persists after conservative treatment, a lumbar epidural blood patch (EBP) without previous leak identification (so-called “blind” EBP) is a widely used initial intervention and may be repeated several times. If EBPs fail, after the CSF leak sites identification using invasive imaging techniques, other therapeutic approaches include: a targeted epidural patch, surgical reduction of dural sac volume, or direct surgical closure. The prognosis is generally good after intervention, but serious complications may occur. More research is needed to better understand SIH pathophysiology to refine imaging modalities and treatment approaches and to evaluate clinical outcomes.

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References

  1. Limaye K, Samant R, Lee RW (2016) Spontaneous intracranial hypotension: diagnosis to management. Acta Neurol Belg 116:119–125

    Article  Google Scholar 

  2. Davidson B, Nassiri F, Mansouri F et al (2017) Spontaneous intracranial hypotension: a review and introduction of an algorithm for management. World Neurosurg 101:343–349

    Article  Google Scholar 

  3. Schievink WI (2006) Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. JAMA 295:2286–2296

    CAS  Article  Google Scholar 

  4. Lin JP, Zhang SD, He FF et al (2017) The status of diagnosis and treatment to intracranial hypotension, including SIH. J Headache Pain 18:4

    Article  Google Scholar 

  5. Brinker T, Stopa E, Morrison J, Klinge P (2014) A new look at cerebrospinal fluid circulation. Fluids Barriers CNS 11:10

    Article  Google Scholar 

  6. Mokri B (2015) Spontaneous intracranial hypotension. Continuum (Minneap Minn) 21:1086–1108

    Google Scholar 

  7. Ducros A, Biousse V (2015) Headache arising from idiopathic changes in CSF pressure. Lancet Neurol 14:655–668

    Article  Google Scholar 

  8. Mokri B, Maher CO, Sencakova D (2002) Spontaneous CSF leaks: underlying disorder of connective tissue. Neurology 58:814–816

    Article  Google Scholar 

  9. Kusnezov NA, Velani SA, Lu DC (2013) Cerebrospinal fluid leak secondary to chiropractic manipulation. Surg Neurol Int 4:S118–S120

    PubMed  PubMed Central  Google Scholar 

  10. Ferrante E, Savino A (2005) Thunderclap headache caused by spontaneous intracranial hypotension. Neurol Sci 26:155–157

    Article  Google Scholar 

  11. Ferrante E, Olgiati E, Sangalli V, Rubino F (2016) Early pain relief from orthostatic headache and hearing changes in spontaneous intracranial hypotension after epidural blood patch. Acta Neurol Belg 1:1. https://doi.org/10.1007/s13760-016-0617-2

    Article  Google Scholar 

  12. Ferrante E, Regna-Gladin C, Arpino I, Citterio A (2010) Spontaneous Intracranial Hypotension Syndrome with hearing loss and pachymeningeal enhancement in the internal acoustic canal: neuroimaging correlations. J Craniofac Surg 21:1660–1661

    Article  Google Scholar 

  13. Ferrante E, Savino A, Brioschi AM et al (1998) Transient oculomotor nerves palsy in spontaneous intracranial hypotension syndrome. J Neurosurg Sci 42:177–179

    CAS  PubMed  Google Scholar 

  14. Schievink WI, Nuño M, Rozen TD et al (2015) Hyperprolactinemia due to spontaneous intracranial hypotension. J Neurosurg 122:1020–1025

    Article  Google Scholar 

  15. Hong M, Shah GV, Adams KM (2002) Spontaneous intracranial hypotension causing reversible frontotemporal dementia. Neurology 58:1285–1287

    CAS  Article  Google Scholar 

  16. Schievink WI, Maya MM (2017) Spinal meningeal diverticula, spontaneous intracranial hypotension, and superficial siderosis. Neurology 88:916–917

    Article  Google Scholar 

  17. Ferrante E, Arpino I, Citterio A, Savino A (2009) Coma resulting from spontaneous intracranial hypotension treated with the epidural blood patch in Trendelenburg position pre-medicated with acetazolamide. Clin Neurol Neurosurg 111:699–702

    Article  Google Scholar 

  18. Ferrante E, Rubino F, Beretta F et al (2018) Treatment and outcome of subdural hematoma in patients with spontaneous intracranial hypotension: a report of 35 cases. Acta Neurol Belg 118:61–70

    Article  Google Scholar 

  19. Headache Classification Committee of the International Headache Society (2018) The International Classification of Headache Disorders, 3rd edition. Cephalalgia 38:1–211

    Google Scholar 

  20. Ferrante E, Regna-Gladin C, Arpino I et al (2013) Pseudo-subarachnoid hemorrhage: a potential imaging pitfall associated with spontaneous intracranial hypotension. Clin Neurol Neurosurg 115:2324–2328

    Article  Google Scholar 

  21. Sencakova D, Mokri B, McClelland RL (2001) The efficacy of epidural blood patch in spontaneous CSF leaks. Neurology 57:1921–1923

    CAS  Article  Google Scholar 

  22. Berroir S, Loisel B, Ducros A, Boukobza M, Tzourio C, Valade D et al (2004) Early epidural blood patch in spontaneous intracranial hypotension. Neurology 63:1950–1951

    CAS  Article  Google Scholar 

  23. Ferrante E, Arpino I, Citterio A, Wetzl R, Savino A et al (2010) Epidural blood patch in Trendelenburg position pre-medicated with acetazolamide to treat spontaneous intracranial hypotension. Eur J Neurol 17:715–719

    CAS  Article  Google Scholar 

  24. Ferrante E, Rubino F, Arpino I et al (2015) Treatment of orthostatic headache from spontaneous intracranial hypotension syndrome: single institutional experience of 326 cases. J Headache Pain 16(Suppl 1):A125

    Article  Google Scholar 

  25. Ferrante E, Rubino F, Beretta F et al (2017) Treatment and outcome of subdural hematoma in patients with spontaneous intracranial hypotension: a report of 35 cases. Acta Neurol Belg. https://doi.org/10.1007/s13760-017-0845-0

    Article  PubMed  Google Scholar 

  26. Kranz PG, Malinzak MD, Amrhein TJ, Gray L (2017) Upadate on the diagnosis and treatment of spontaneous intracranial hypotension. Curr Pain Headache Rep 21:37

    Article  Google Scholar 

  27. Takai K, Niimura M, Hongo H et al (2019) Disturbed consciousness and coma: diagnosis and management of intracranial hypotension caused by a spinal cerebrospinal fluid leak. World Neurosurg 121:e700–e711

    Article  Google Scholar 

  28. Ferrante E, Rubino F, Prone V et al (2016) Cerebral superficial siderosis following chronic intracranial hypotension. Neurol Sci 37:S3

    Article  Google Scholar 

  29. Schievink WI, Maya MM, Chow W, Louy C (2007) Reversible cerebral vasoconstriction in spontaneous intracranial hypotension. Headache 47:284–287

    Article  Google Scholar 

  30. Schievink WI, Maya MM, Moser FG (2017) Digital subtraction myelography in the investigation of post-dural puncture headache in 27 patients: technical note. J Neurosurg Spine 26:760–764

    Article  Google Scholar 

  31. Kranz PG, Amrhein TJ, Schievink WI, Karikari IO, Gray L et al (2016) The “hyperdense paraspinal vein” sign: a marker of CSF-venous fistula. AJNR Am J Neuroradiol 37:1379–1381

    CAS  Article  Google Scholar 

  32. Ferrante E, Rubino F (2014) Orthostatic headache without intracranial hypotension: a headache due to psychiatric disorder? Headache 54:1056–1057

    Article  Google Scholar 

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Correspondence to Enrico Ferrante.

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Enrico Ferrante declares that he has no conflict of interest. Michele Trimboli declares that he has no conflict of interest. Fabio Rubino declares that he has no conflict of interest.

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Ferrante, E., Trimboli, M. & Rubino, F. Spontaneous intracranial hypotension: review and expert opinion. Acta Neurol Belg 120, 9–18 (2020). https://doi.org/10.1007/s13760-019-01166-8

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Keywords

  • Orthostatic headache
  • Spontaneous intracranial hypotension
  • CSF leak
  • Cerebrospinal fluid
  • Epidural blood patch