Smarter Decisions,
Better Care

UpToDate synthesizes the most recent medical information into evidence-based practical recommendations clinicians trust to make the right point-of-care decisions.

  • Rigorous editorial process: Evidence-based treatment recommendations
  • World-Renowned physician authors: over 5,100 physician authors and editors around the globe
  • Innovative technology: integrates into the workflow; access from EMRs

Choose from the list below to learn more about subscriptions for a:


Subscribers log in here


Headache attributed to spontaneous intracranial hypotension: Treatment and prognosis

INTRODUCTION

The production, absorption, and flow of cerebrospinal fluid (CSF) play key roles in the dynamics of intracranial pressure. Alterations in CSF pressure can lead to neurologic symptoms, the most common being headache. Most often, the headaches associated with low CSF pressure are orthostatic and occur after lumbar puncture, but similar headaches occur with spontaneous low CSF pressure due to spinal CSF leaks, and with CSF shunt overdrainage [1,2].

Headache attributed to spontaneous intracranial hypotension is being recognized with increasing frequency. Orthostatic headache, low CSF pressure, and diffuse meningeal enhancement on brain magnetic resonance imaging (MRI) are the major features of the classic syndrome. However, some cases have nonorthostatic headache, normal CSF pressure, or no evidence of diffuse meningeal enhancement [2]. As our understanding of this syndrome is still evolving, the terms "low CSF pressure headache," "spontaneous intracranial hypotension," "spontaneous CSF leak," "CSF hypovolemia," and "CSF volume depletion" tend to be used interchangeably.

This topic will review the treatment and prognosis of headache attributed to spontaneous intracranial hypotension. Other aspects of this disorder are discussed separately. (See "Headache attributed to spontaneous intracranial hypotension: Pathophysiology, clinical features, and diagnosis".)

Post-lumbar puncture headache is reviewed elsewhere. (See "Post-lumbar puncture headache".)

DIAGNOSIS

The diagnosis of headache attributed to spontaneous intracranial hypotension is reviewed here briefly and discussed in detail separately (see "Headache attributed to spontaneous intracranial hypotension: Pathophysiology, clinical features, and diagnosis", section on 'Diagnosis'). The diagnosis should be considered in patients who present with positional orthostatic headache, with or without associated symptoms, perhaps in the setting of minor trauma, and in the absence of a history of dural puncture or other cause of CSF fistula. Confirmation of the diagnosis requires evidence of low CSF pressure by lumbar puncture, and/or evidence of CSF leakage on imaging with MRI (image 1 and image 2), computed tomographic myelography or radioisotope cisternography.

         

Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: Nov 2014. | This topic last updated: May 29, 2014.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2014 UpToDate, Inc.
References
Top
  1. Schievink WI, Reimer R, Folger WN. Surgical treatment of spontaneous intracranial hypotension associated with a spinal arachnoid diverticulum. Case report. J Neurosurg 1994; 80:736.
  2. Mokri B. Spontaneous cerebrospinal fluid leaks: from intracranial hypotension to cerebrospinal fluid hypovolemia--evolution of a concept. Mayo Clin Proc 1999; 74:1113.
  3. Schievink WI. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. JAMA 2006; 295:2286.
  4. Hoffmann J, Goadsby PJ. Update on intracranial hypertension and hypotension. Curr Opin Neurol 2013; 26:240.
  5. Marcelis J, Silberstein SD. Spontaneous low cerebrospinal fluid pressure headache. Headache 1990; 30:192.
  6. Lipman IJ. Primary intracranial hypotension: the syndrome of spontaneous low cerebospinal fluid pressure with traction headache. Dis Nerv Syst 1977; 38:212.
  7. Mokri B. Low cerebrospinal fluid pressure syndromes. Neurol Clin 2004; 22:55.
  8. Sencakova D, Mokri B, McClelland RL. The efficacy of epidural blood patch in spontaneous CSF leaks. Neurology 2001; 57:1921.
  9. Berroir S, Loisel B, Ducros A, et al. Early epidural blood patch in spontaneous intracranial hypotension. Neurology 2004; 63:1950.
  10. Mokri B. Spontaneous CSF leaks: low CSF volume syndromes. Neurol Clin 2014; 32:397.
  11. Cho KI, Moon HS, Jeon HJ, et al. Spontaneous intracranial hypotension: efficacy of radiologic targeting vs blind blood patch. Neurology 2011; 76:1139.
  12. Crul BJ, Gerritse BM, van Dongen RT, Schoonderwaldt HC. Epidural fibrin glue injection stops persistent postdural puncture headache. Anesthesiology 1999; 91:576.
  13. Schievink WI, Maya MM, Moser FM. Treatment of spontaneous intracranial hypotension with percutaneous placement of a fibrin sealant. Report of four cases. J Neurosurg 2004; 100:1098.
  14. Schievink WI, Georganos SA, Maya MM, et al. Anaphylactic reactions to fibrin sealant injection for spontaneous spinal CSF leaks. Neurology 2008; 70:885.
  15. Beierlein W, Scheule AM, Dietrich W, Ziemer G. Forty years of clinical aprotinin use: a review of 124 hypersensitivity reactions. Ann Thorac Surg 2005; 79:741.
  16. Lay CL, Campbell JK, Mokri B. Low cerebrospinal fluid pressure headache. In: Headache, Goadsby PJ, Silberstein SD (Eds), Butterworth-Heinemann, Boston 1997. p.355.
  17. Binder DK, Dillon WP, Fishman RA, Schmidt MH. Intrathecal saline infusion in the treatment of obtundation associated with spontaneous intracranial hypotension: technical case report. Neurosurgery 2002; 51:830.
  18. Schievink WI, Morreale VM, Atkinson JL, et al. Surgical treatment of spontaneous spinal cerebrospinal fluid leaks. J Neurosurg 1998; 88:243.
  19. Rando TA, Fishman RA. Spontaneous intracranial hypotension: report of two cases and review of the literature. Neurology 1992; 42:481.
  20. Schievink WI, Maya MM, Louy C. Cranial MRI predicts outcome of spontaneous intracranial hypotension. Neurology 2005; 64:1282.