Background Spontaneous intracranial hypotension (SIH) has become a well-recognized syndrome. However, diagnosis of SIH is still challenging. The problem with SIH is that the precise mechanism of cerebrospinal fluid (CSF) leakage remains largely unknown and there is no definite diagnostic criterion in the imaging.
Methods The clinical findings of our ten cases and 301 literature reports on SIH were investigated in a retrospective analysis to clarify the pathophysiology of CSF leakage, correlate the findings of imaging studies and determine the most adequate diagnostic criteria.
Results The events precede symptoms of SIH were categorized as traumatic, secondary and strictly spontaneous (62%). The location of the spinal CSF leak remains undetectable in approximately 50% of cases reported. In 93% of patients, the CSF leakage sites were detected at the cervical or thoracic level of the spine. On recent MRI studies, 88% of patients showed spinal epidural fluid collections that most likely represent CSF leakage. MR myelography using heavily T2-weighted fast-spin-echo sequence can clearly demonstrate the site of CSF leakage. Although numerous treatment options are available, none of the treatments have been evaluated by randomized clinical trials. In 48% of papers, autologous epidural blood patch (EBP) was the treatment of choice in patients who have failed initial conservative management. Forty-nine percent of patients showed relief of symptoms after up to three repeated EBPs.
Conclusion We propose new diagnostic criteria of SIH to avoid misdiagnosis.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
References
Chung SJ, Kim JS, Lee MC (2000) Syndrome of cerebral spinal fluid hypovolemia: clinical and imaging features and outcome. Neurology 55:1321–1327
Headache Classification Subcommittee of the International Headache Society (2004) The international classification of headache disorders, 2nd edition. Cephalgia 24(Suppl 1):1–160
Ishihara S, Otani N, Shima K (2003) Spontaneous intracranial hypotension (SIH): the early appearance of urinary bladder activity in RI cisternography is a pathognomonic sign of SIH? Acta Neurochir 86(Suppl):587–589
Ishihara S, Fukui S, Otani N, Miyazawa T, Ohnuki A, Kato H, Tsuzuki N, Nawashiro H, Shima K (2001) Evaluation of spontaneous intracranial hypotension: assessment on ICP monitoring and radiological imaging. Br J Neurosurg 15:239–241
Matsumura A, Anno I, Kimura H, Ishikawa E, Nose T (2000) Diagnosis of spontaneous intracranial hypotension by using magnetic resonance myelography. J Neurosurg 92:873–876
Mokri B, Piepgras DG, Miller GM (1997) Syndrome of orthostatic headaches and diffuse pachymeningeal gadolinium enhancement. Mayo Clin Proc 72:400–413
Rando TA, Fishman RA (1992) Spontaneous intracranial hypotension: report of two cases and review of the literature. Neurology 42:481–487
River Y, Schwartz A, Gomori JM, Soffer D, Siegal T (1996) Clinical significance of diffuse dural enhancement detected by magnetic resonance imaging. J Neurosurg 85:777–783
Shievink WI (2000) Spontaneous spinal cerebrospinal fluid leaks: a review. Neurosurg Focus 9:8
Schievievink WI (2006) Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. JAMA 295:2286– 2296
Author information
Authors and Affiliations
Editor information
Rights and permissions
Copyright information
© 2008 Springer-Verlag/Wien
About this paper
Cite this paper
Shima, K., Ishihara, S., Tomura, S. (2008). Pathophysiology and diagnosis of spontaneous intracranial hypotension. In: Steiger, H.J. (eds) Acta Neurochirurgica Supplements. Acta Neurochirurgica Supplementum, vol 102. Springer, Vienna. https://doi.org/10.1007/978-3-211-85578-2_31
Download citation
DOI: https://doi.org/10.1007/978-3-211-85578-2_31
Publisher Name: Springer, Vienna
Print ISBN: 978-3-211-85577-5
Online ISBN: 978-3-211-85578-2
eBook Packages: MedicineMedicine (R0)