Diagnostics and Treatment of Spontaneous Intracranial Hypotension

Abstract

Objective: Intracranial hypotension is a frequently misdiagnosed syndrome which is caused by reduced intracranial cerebrospinal fluid (CSF) pressure due to spontaneous spinal CSF leakage. We present our series of intracranial hypotension regarding especially the required diagnostic imaging and the treatment.

Methods: A retrospective analysis was performed on 8 patients (5 males, 3 females, mean age 49 years) with postural and non-postural headache due to spinal CSF collection.

Results: Cranial MRI showed diffuse pachymeningeal gadolinium enhancement in all cases. CSF leakage detected by gadolinium-enhanced MR cisternography could be either diffuse (n=5) or precisely located around a dural tear (n=3). All but one leakages were located at the thoracic spine. In 6 patients 40–65 mL of blood were injected through epidurally placed drainages. In 1 patient, a dural tear was sealed with fibrin glue and fat. One patient refused surgical intervention. One epidural haematoma had to be revised. 5 of 7 patients showed excellent results.

Conclusion: Gadolinium-enhanced MR cisternography best revealed CSF leaks. In the majority of patients with spontaneous intracranial hypotension, complete recovery may be achieved via a midthoracic epidural blood patch with minimal complications.

headache - cerebrospinal fluid leak - intracranial hypotension - MR cisternography

References

• 1 Aoki N, Sakai T, Oikawa A. Spontaneous intracranial hypotension associated with subdural hematoma: diagnostic usefulness of percutaneous subdural tapping and magnetic resonance imaging.  Acta Neurochir (Wien). 1998;  140 47-49 ; discussion 49-50
• 2 Binder DK, Sarkissian V, Dillon WP. et al . Spontaneous intracranial hypotension associated with transdural thoracic osteophyte reversed by primary. dural repair.  Case report. J Neurosurg Spine. 2005;  2 614-618
• 3 Ferrante E, Savino A, Sances G. et al . Spontaneous intracranial hypotension syndrome: report of twelve cases.  Headache. 2004;  44 615-622
• 4 Nowak DA, Rodiek SO, Zinner J. et al . Broadening the clinical spectrum: unusual presentation of spontaneous cerebrospinal fluid hypovolemia.  Case report. J Neurosurg. 2003;  98 903-907
• 5 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 1098-1100
• 6 Schievink WI, Tourje J. Upright MRI in spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension.  Headache. 2007;  47 1345-1346
• 7 Atkinson JL, Weinshenker BG, Miller GM. et al . Acquired Chiari I malformation secondary to spontaneous spinal cerebrospinal fluid leakage and chronic intracranial hypotension syndrome in seven cases.  J Neurosurg. 1998;  88 237-242
• 8 Chiapparini L, Ciceri E, Nappini S. et al . Headache and intracranial hypotension: neuroradiological findings.  Neurol Sci. 2004;  25 (S 03) S138-S141
• 9 Spitzer C, Wessels T, Block F. Extraordinary high cerebrospinal fluid protein in two cases of intracranial hypotension syndrome.  Nervenarzt. 2004;  75 1222-1225
• 10 Albayram S, Kilic F, Ozer H. et al . Gadolinium-enhanced MR cisternography to evaluate dural leaks in intracranial hypotension syndrome.  AJNR Am J Neuroradiol. 2008;  29 116-121
• 11 Platz J, Glucker T, Gratzl O. et al . Spontaneous intracranial hypotension: case report with subdural hematomas, steroid dependency and clinical improvement after myelography.  Zentralbl Neurochir. 2007;  68 87-90
• 12 Milledge JT, Ades LC, Cooper MG. et al . Severe spontaneous intracranial hypotension and Marfan syndrome in an adolescent.  J Paediatr Child Health. 2005;  41 68-71
• 13 Mokri B, Maher CO, Sencakova D. Spontaneous CSF leaks: underlying disorder of connective tissue.  Neurology. 2002;  58 814-816
• 14 Rando TA, Fishman RA. Spontaneous intracranial hypotension: report of two cases and review of the literature.  Neurology. 1992;  42 481-487
• 15 Wang YF, Fuh JL, Lirng JF. et al . Spontaneous intracranial hypotension with isolated cortical vein thrombosis and subarachnoid haemorrhage.  Cephalalgia. 2007;  27 1413-1417
• 16 Wang LP, Schmidt JF. Central nervous side effects after lumbar puncture. A review of the possible pathogenesis of the syndrome of postdural puncture headache and associated symptoms.  Dan Med Bull. 1997;  44 79-81
• 17 Watanabe A, Horikoshi T, Uchida M. et al . Diagnostic value of spinal MR imaging in spontaneous intracranial hypotension syndrome.  AJNR Am J Neuroradiol. 2009;  30 147-151
• 18 Chung SJ, Kim JS, Lee MC. Syndrome of cerebral spinal fluid hypovolemia: clinical and imaging features and outcome.  Neurology. 2000;  55 1321-1327
• 19 Richard S, Kremer S, Lacour JC. et al . Cerebral venous thrombosis caused by spontaneous intracranial hypotension: two cases.  Eur J Neurol. 2007;  14 1296-1298
• 20 Bai J, Yokoyama K, Kinuya S. et al . Radionuclide cisternography in intracranial hypotension syndrome.  Ann Nucl Med. 2002;  16 75-78
• 21 Sayer FT, Bodelsson M, Larsson EM. et al . Spontaneous intracranial hypotension resulting in coma: case report.  Neurosurgery. 2006;  59 E204 ; discussion E204

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