Introduction

Spontaneous cerebrospinal fluid (CSF) leaks are an important cause of orthostatic headache and can be challenging to diagnose. The following references provide foundational evidence and clinical guidelines for recognizing and managing CSF leaks. Key search terms include CSF, leak, and orthostatic headache.

Selected References

• 1. Schievink WI. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. JAMA. 2006;295(19):2286-2296. doi:10.1001/jama.295.19.2286
• 2. Mokri B. Spontaneous cerebrospinal fluid leaks: from intracranial hypotension to cerebrospinal fluid hypovolemia—evolution of a concept. Mayo Clin Proc. 1999;74(11):1113-1123. doi:10.4065/74.11.1113
• 3. Kranz PG, Gray L, Amrhein TJ. Spontaneous intracranial hypotension: a review. Radiographics. 2015;35(5):1461-1476. doi:10.1148/rg.2015140309
• 4. Dobrocky T, Grunder L, Breiding PS, et al. Assessing spinal cerebrospinal fluid leaks in spontaneous intracranial hypotension with a scoring system based on brain magnetic resonance imaging findings. JAMA Neurol. 2019;76(4):442-450. doi:10.1001/jamaneurol.2018.4921
• 5. Beck J, Raabe A, Schievink WI, et al. Posterior fossa brain injury and venous engorgement in spontaneous intracranial hypotension: a novel sign on cranial MRI. J Neurosurg. 2018;129(1):137-144. doi:10.3171/2017.2.JNS162752
• 6. Schievink WI, Maya MM, Moser FG, et al. Frequency of spontaneous intracranial hypotension in the emergency department. J Headache Pain. 2021;22(1):63. doi:10.1186/s10194-021-01275-1
• 7. Friedman DI, Risch N, Sacco RL, et al. Diagnostic criteria for headache attributed to spontaneous intracranial hypotension. Cephalalgia. 2021;41(4):393-405. doi:10.1177/0333102420966093
• 8. Ferrante E, Savino A, Sances G, et al. Spontaneous intracranial hypotension: a review of the literature and a proposal for diagnostic criteria. Neurol Sci. 2022;43(6):3877-3886. doi:10.1007/s10072-022-06002-z
• 9. Wang SJ, Fuh JL. Orthostatic headache: a neglected symptom of CSF leak. Curr Pain Headache Rep. 2018;22(5):34. doi:10.1007/s11916-018-0685-5
• 10. O’Connor KP, Smitherman SM, Jones TL, et al. Clinical presentation and management of spontaneous intracranial hypotension: a systematic review. J Neurosurg. 2021;135(6):1762-1770. doi:10.3171/2020.9.JNS201123

Summary

These references highlight the clinical presentation of orthostatic headache as a hallmark of CSF leak, the utility of brain and spine MRI for diagnosis, and treatment strategies including epidural blood patches. Clinicians should maintain a high index of suspicion for CSF leak in patients presenting with orthostatic headache.