Current Pain and Headache Reports

, Volume 11, Issue 1, pp 56–61

Spontaneous intracranial hypotension



Spontaneous intracranial hypotension (SIH) is caused by leakage of cerebrospinal fluid (CSF), with resultant CSF hypovolemia and intracranial hypotension. Although in some patients SIH may be preceded by minor trauma, it often occurs in the absence of any identifiable initiating event. Orthostatic headache is the primary clinical manifestation, usually accompanied by one or more other symptoms of SIH. Diagnosis can be reached by a combination of imaging studies, which may include MRI with gadolinium, nuclear cisternography, and myelography. Treatment ranges from conservative management, such as bed rest and hydration, to invasive procedures, such as lumbar puncture with autologous blood patch, CT-guided fibrin glue injection at the site of the leak, and open surgical intervention. Outcomes vary from complete resolution of CSF leak with alleviation of symptoms to continued and/or recurrent leaks with chronic unremitting symptomatology.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References and Recommended Reading

  1. 1.
    Schievink WI, Gordon OK, Tourje J: Connective tissue disorders with spontaneous cerebrospinal fluid leaks and intracranial hypotension: a prospective study. Neurosurgery 2004, 54:65–71.PubMedCrossRefGoogle Scholar
  2. 2.
    Mokri B, Maher CO, Sencakova D: Spontaneous CSF leaks: underlying disorders of connective tissue. Neurology 2002, 58:814–816.PubMedGoogle Scholar
  3. 3.
    Mokri B: Low cerebrospinal fluid pressure syndromes. Neurol Clin N Am 2004, 22:55–74.Google Scholar
  4. 4.
    Headache Classification Committee of the International Headache Society. The International classification of headache disorders. Cephalalgia 2004, 24(Suppl 1):1–151.Google Scholar
  5. 5.
    Mokri B, Atkinson JLK, Piepgras DG: Absent headache despite CSF volume depletion (intracranial hypotension). Neurology 2000, 55:573–575.PubMedGoogle Scholar
  6. 6.
    Mokri B: Spontaneous CSF leaks mimicking benign exertional headaches. Cephalalgia 2002, 22:780–783.PubMedCrossRefGoogle Scholar
  7. 7.
    Scheivink WI, Wijdicks EFM, Meyer FB, Sonntag VK: Spontaneous intracranial hypotension mimicking aneurysmal subarachnoid hemorrhage. Neurosurgery 2001, 48:513–517.CrossRefGoogle Scholar
  8. 8.
    Mokri B: Headaches caused by decreased intracranial pressure: diagnosis and management. Curr Opin Neurol 2003, 16:319–326.PubMedCrossRefGoogle Scholar
  9. 9.
    Albayram S, Wasserman BA, Yousem DM, Wityk R: Intracranial hypotension as a cause of radiculopathy from cervical epidural venous engorgement. Case report. AJNR Am J Neuroradiol 2002, 23:618–621.PubMedGoogle Scholar
  10. 10.
    Hong M, Shah GV, Adams KM, et al.: Spontaneous intracranial hypotension causing reversible frontotemporal dementia. Neurology 2002, 58:1285–1287.PubMedGoogle Scholar
  11. 11.
    Evans RW, Mokri B: Spontaneous intracranial hypotension resulting in coma. Headache 2002, 42:159–160.CrossRefGoogle Scholar
  12. 12.
    Schievink WI, Maya MM, Moser FG, Tourje J: Spectrum of subdural fluid collections in spontaneous intracranial hypotension. J Neurosurg 2005, 103:608–613.PubMedGoogle Scholar
  13. 13.
    Mokri B, Piepgras DG, Miller GM: Syndrome of orthostatic headaches and diffuse pachymeningeal gadolinium enhancement. Mayo Clin Proc 1997, 72:400–413.PubMedGoogle Scholar
  14. 14.
    Rice DH: Cerebrospinal fluid rhinorrhea: diagnosis and treatment. Curr Opin Otolaryngol Head Neck Surg 2003, 11:19–22.PubMedCrossRefGoogle Scholar
  15. 15.
    Zlab MK, Moore GF, Daly DT, Yonkers AJ: Cerebrospinal fluid rhinorrhea: a review of the literature. Ear Nose Throat J 1992, 71:314–317.PubMedGoogle Scholar
  16. 16.
    Lund VJ: Endoscopic management of cerebrospinal fluid leaks. Am J Rhinol 2002, 16:17–23.PubMedGoogle Scholar
  17. 17.
    Eros EJ, Dodick DW, Nelson KD: Orthostatic headache syndrome with CSF leak secondary to bony pathology of the cervical spine. Cephalalgia 2002, 22:439–443.CrossRefGoogle Scholar
  18. 18.
    Vishteh AG, Schievink WI, Baskin JJ, Sonntag VKH: Cervical bony spur presenting with spontaneous intracranial hypotension. Case report. J Neurosurg 1998, 89:483–484.PubMedGoogle Scholar
  19. 19.
    Schievink WI: Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. JAMA 2006, 295:2286–2296.PubMedCrossRefGoogle Scholar
  20. 20.
    Portier F, Minteguiaga C, Racy E, et al.: Spontaneous intracranial hypotension: a rare cause of labyrinthe hydrops. Ann Otol Rhinol Laryngol 2002, 111:817–820.PubMedGoogle Scholar
  21. 21.
    Lin WC, Lirng JF, Fuh JL, et al.: MR findings of spontaneous intracranial hypotension. Acta Radiol 2002, 43:249–255.PubMedCrossRefGoogle Scholar
  22. 22.
    Berroir S, Loisel B, Ducros A, et al.: Early epidural blood patch in spontaneous intracranial hypotension. Neurology 2004, 63:1950–1951.PubMedGoogle Scholar
  23. 23.
    Schievink WI, Maya MM, Louy C: Cranial MRI predicts outcome of spontaneous intracranial hypotension. Neurology 2005, 64:1282–1284.PubMedGoogle Scholar
  24. 24.
    Chen CJ, Lee TH, Hsu HL, et al.: Spinal MR findings in spontaneous intracranial hypotension. Neuroradiology 2002, 44:996–1003.PubMedCrossRefGoogle Scholar
  25. 25.
    Molins A, Alvarez J, Sumalla J, et al.: Cisternographic pattern of spontaneous liquoral hypotension. Cephalalgia 1990, 10:59–65.PubMedCrossRefGoogle Scholar
  26. 26.
    Grantham VV, Blakley B, Winn J: Technical review and considerations for a cerebrospinal fluid leakage study. J Nucl Med Technol 2006, 34:48–51.PubMedGoogle Scholar
  27. 27.
    Matsumura A, Anno I, Kimura H, et al.: Diagnosis of spontaneous intracranial hypotension by using magnetic resonance myelography. J Neurosurg 2000, 92:873–876.PubMedGoogle Scholar
  28. 28.
    Luetmer PH, Mokri B: Dynamic CT myelography: a technique for localizing high-flow spinal cerebrospinal fluid leaks. AJNR Am J Neuroradiol 2003, 24:1711–1714.PubMedGoogle Scholar
  29. 29.
    Zlab MK, Moore GF, Daly DT, Yonkers AJ: Cerebrospinal fluid rhinorrhea: a review of the literature. Ear Nose Throat J 1992, 71:314–317.PubMedGoogle Scholar
  30. 30.
    Hull HF, Morrow G: Glucorrhea revisited. JAMA 1975, 234:1052–1053.PubMedCrossRefGoogle Scholar
  31. 31.
    Mokri B: Spontaneous low cerebrospinal pressure/volume headaches. Curr Neurol Neurosci Rep 2004, 4:117–124.PubMedGoogle Scholar
  32. 32.
    Karst M, Hollenhorst J, Fink M, Conrad I: Computerized tomography-guided epidural blood patch in the treatment of spontaneous low cerebrospinal fluid pressure headache. Acta Anaesthesiol Scand 2001, 45:649–651.PubMedCrossRefGoogle Scholar
  33. 33.
    Duffy PJ, Crosby ET: The epidural blood patch. Resolving the controversies. Can J Anaesth 1999, 46:878–886.PubMedCrossRefGoogle Scholar
  34. 34.
    Sencakova D, Mokri B, McClelland RL: The efficacy of epidural blood patch in spontaneous CSF leaks. Neurology 2001, 57:1921–1923.PubMedGoogle Scholar
  35. 35.
    Schievink WI, Maya MM, Moser FM: Treatment of spontaneous intracranial hypotension with percutaneous placement of a fibrin sealant. J Neurosurg 2004, 100:1098–1100.PubMedGoogle Scholar
  36. 36.
    Gladstone JP, Nelson K, Patel N, Dodick DW: Spontaneous CSF leak treated with percutaneous CT-guided fibrin glue. Neurology 2005, 64:1818–1819.PubMedCrossRefGoogle Scholar
  37. 37.
    Schievink WI, Morreale VM, Atkinson JL, et al.: Surgical treatment of spontaneous spinal cerebrospinal fluid leaks. J Neurosurg 1998, 88:243–246.PubMedGoogle Scholar
  38. 38.
    Kong DS, Park K, Nam DH, et al.: Clinical features and long-term results of spontaneous intracranial hypotension. Neurosurgery 2005, 57:91–96.PubMedCrossRefGoogle Scholar
  39. 39.
    Schievink WI, Maya MM, Riedinger M: Recurrent spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension: a prospective study. J Neurosurg 2003, 99:840–842.PubMedCrossRefGoogle Scholar

Copyright information

© Current Medicine Group LLC 2007

Authors and Affiliations

  1. 1.Department of NeurologyMayo Clinic College of MedicineScottsdaleUSA

Personalised recommendations