Update on spontaneous intracranial hypotension
DOI:
https://doi.org/10.48208/HeadacheMed.2026.10Keywords:
Spontaneous intracranial hypotension, Cerebrospinal fluid leak, Orthostatic headache, CT myelography, Epidural blood patchAbstract
Background
Spontaneous intracranial hypotension (SIH) is a secondary headache disorder caused by spontaneous cerebrospinal fluid (CSF) leakage, typically from dural tears, meningeal diverticula, or CSF venous fistulas, in the absence of preceding trauma or procedures. Although increasingly recognized, SIH remains underdiagnosed and frequently mistaken for primary headache syndromes.
Methods
This narrative review synthesizes current evidence on the epidemiology, pathophysiology, clinical manifestations, neuroimaging features, complications, and management of SIH. Recent literature and expert consensus were analyzed to integrate advances in imaging-based diagnosis and leak type specific therapeutic strategies.
Results
SIH arises from low CSF volume rather than true hypotension, leading to brain descent, venous engorgement, and the characteristic MRI features summarized by the SEEPS mnemonic: subdural collections, pachymeningeal enhancement, venous engorgement, pituitary enlargement, and brain sagging. Orthostatic headache is the hallmark symptom, though up to 15% of patients lack clear positional features. Associated symptoms include vestibulocochlear disturbances, neck or interscapular pain, cranial nerve palsies, and cognitive or motor dysfunction. Brain and whole spine MRI are first line diagnostic tools. MR myelography distinguishes SLEC positive (types 1 and 2) from SLEC negative (type 3) leaks, guiding targeted myelography with dynamic CT or digital subtraction techniques for leak localization. Treatment should be tailored to leak type and site: epidural blood or fibrin patching for dural and diverticular leaks, and transvenous embolization for CSF venous fistulas. Surgical repair is reserved for refractory or anatomically complex cases. Reported complications include subdural hematoma, cerebral venous thrombosis, superficial siderosis, and bibrachial amyotrophy.
Conclusions
SIH represents a heterogeneous spinal disorder with intracranial manifestations. Early recognition, standardized imaging algorithms, and multidisciplinary management are crucial to improving outcomes. Ongoing research into imaging biomarkers, predictors of response, and consensus based care pathways will help advance precision medicine in SIH.
Downloads
References
1. Schievink WI. Spontaneous Intracranial Hypotension. New England Journal of Medicine 2021;385:2173–8. Doi:10.1056/NEJMra2101561. DOI: https://doi.org/10.1056/NEJMra2101561
2. Zander C, Wolf K, El Rahal A, Volz F, Beck J, Urbach H, et al. Spontaneous intracranial hypotension – a spinal disease. RöFo - Fortschritte Auf Dem Gebiet Der Röntgenstrahlen Und Der Bildgebenden Verfahren 2025;197:44–54. Doi:10.1055/a-2318-8994. DOI: https://doi.org/10.1055/a-2318-8994
3. Palermo M, Sturiale CL, D’Arrigo S, Trevisi G. Targeted Versus NonTargeted Epidural Blood Patch for Spontaneous Intracranial Hypotension: A Systematic Review and Meta‐Analysis. Eur J Neurol 2025;32. Doi:10.1111/ene.70239. DOI: https://doi.org/10.1111/ene.70239
4. Kranz PG, Tanpitukpongse TP, Choudhury KR, Amrhein TJ, Gray L. How common is normal cerebrospinal fluid pressure in spontaneous intracranial hypotension? Cephalalgia 2016;36:1209–17. Doi:10.1177/0333102415623071. DOI: https://doi.org/10.1177/0333102415623071
5. Goddu SKG, Adiga CP, Kumar S, Goolahally LN, Kumar S. Spontaneous Intracranial Hypotension: A Review of Neuroimaging and Current Concepts. Indian Journal of Radiology and Imaging 2024;34:128–38. Doi:10.1055/s-0043-1774301. DOI: https://doi.org/10.1055/s-0043-1774301
6. Schievink WI. Misdiagnosis of Spontaneous Intracranial Hypotension. Arch Neurol 2003;60:1713. Doi:10.1001/archneur.60.12.1713. DOI: https://doi.org/10.1001/archneur.60.12.1713
7. D’Antona L, Jaime Merchan MA, Vassiliou A, Watkins LD, Davagnanam I, Toma AK, et al. Clinical Presentation, Investigation Findings, and Treatment Outcomes of Spontaneous Intracranial Hypotension Syndrome. JAMA Neurol 2021;78:329. Doi:10.1001/jamaneurol.2020.4799. DOI: https://doi.org/10.1001/jamaneurol.2020.4799
8. Volz F, Wolf K, Fung C, Carroll I, Lahmann C, Lützen N, et al. Impact of Spinal CSF Leaks on Quality of Life and Mental Health and Long-Term Reversal by Surgical Closure. Neurol Clin Pract 2024;14. Doi:10.1212/CPJ.0000000000200272. DOI: https://doi.org/10.1212/CPJ.0000000000200272
9. Kapan A, Waldhör T, Wöber C. Assessing the effects of spontaneous intracranial hypotension on quality of life, work ability and disability. Wien Klin Wochenschr 2025;137:148–62. Doi:10.1007/s00508-024-02423-4. DOI: https://doi.org/10.1007/s00508-024-02423-4
10. Headache Classification Committee of the International Headache Society (IHS) The International Classification of Headache Disorders, 3rd edition. Cephalalgia 2018;38:1–211. Doi:10.1177/0333102417738202. DOI: https://doi.org/10.1177/0333102417738202
11. Olesen J. Preface to the Second Edition. Cephalalgia 2004;24:9–10. Doi:10.1111/j.1468-2982.2003.00824.x. DOI: https://doi.org/10.1111/j.1468-2982.2003.00824.x
12. Rau JC, Cutsforth-Gregory JK. Clinical Features and Diagnosis of Spontaneous Intracranial Hypotension. Continuum (N Y) 2025;31:644–67. Doi:10.1212/cont.0000000000001566. DOI: https://doi.org/10.1212/cont.0000000000001566
13. Mehta D, Cheema S, Glover S, Qureshi AM, Davagnanam I, Kamourieh S, et al. Defining the typical characteristics of orthostatic headache in patients with spontaneous intracranial hypotension. Cephalalgia 2025;45. Doi:10.1177/03331024241308154. DOI: https://doi.org/10.1177/03331024241308154
14. Wicklund MR, Mokri B, Drubach DA, Boeve BF, Parisi JE, Josephs KA. Frontotemporal brain sagging syndrome. Neurology 2011;76:1377–82. Doi:10.1212/WNL.0b013e3182166e42. DOI: https://doi.org/10.1212/WNL.0b013e3182166e42
15. Schievink WI, Maya MM, Moser FG, Tourje J. Spectrum of subdural fluid collections in spontaneous intracranial hypotension. J Neurosurg 2005;103:608–13. Doi:10.3171/jns.2005.103.4.0608. DOI: https://doi.org/10.3171/jns.2005.103.4.0608
16. Schievink WI, Maya MM, Harris J, Galvan J, Taché RB, Nuño M. Infratentorial Superficial Siderosis and Spontaneous Intracranial Hypotension. Ann Neurol 2023;93:64–75. Doi:10.1002/ana.26521. DOI: https://doi.org/10.1002/ana.26521
17. Schievink WI, Maya M, Moser F, Nuño M. Long-term Risks of Persistent Ventral Spinal CSF Leaks in SIH. Neurology 2021;97. Doi:10.1212/WNL.0000000000012786. DOI: https://doi.org/10.1212/WNL.0000000000012786
18. Farb RI, Nicholson PJ, Peng PW, Massicotte EM, Lay C, Krings T, et al. Spontaneous Intracranial Hypotension: A Systematic Imaging Approach for CSF Leak Localization and Management Based on MRI and Digital Subtraction Myelography. American Journal of Neuroradiology 2019. Doi:10.3174/ajnr.A6016. DOI: https://doi.org/10.3174/ajnr.A6016
19. Mokri B. Spontaneous Cerebrospinal Fluid Leaks: From Intracranial Hypotension to Cerebrospinal Fluid Hypovolemia—Evolution of a Concept. Mayo Clin Proc 1999;74:1113–23. Doi:10.4065/74.11.1113. DOI: https://doi.org/10.4065/74.11.1113
20. Callen AL, Pattee J, Thaker AA, Timpone VM, Zander DA, Turner R, et al. Relationship of Bern Score, Spinal Elastance, and Opening Pressure in Patients With Spontaneous Intracranial Hypotension. Neurology 2023;100. Doi:10.1212/WNL.0000000000207267. DOI: https://doi.org/10.1212/WNL.0000000000207267
21. Cheema S, Anderson J, Angus-Leppan H, Armstrong P, Butteriss D, Carlton Jones L, et al. Multidisciplinary consensus guideline for the diagnosis and management of spontaneous intracranial hypotension. J Neurol Neurosurg Psychiatry 2023;94:835–43. Doi:10.1136/jnnp-2023-331166.
22. Carlton Jones L, Butteriss D, Scoffings D. Spontaneous intracranial hypotension: the role of radiology in diagnosis and management. Clin Radiol 2022;77:e181–94. Doi:10.1016/j.crad.2021.11.007. DOI: https://doi.org/10.1016/j.crad.2021.11.007
23. Tosaka M, Sato N, Fujimaki H, Tanaka Y, Kagoshima K, Takahashi A, et al. Diffuse Pachymeningeal Hyperintensity and Subdural Effusion/Hematoma Detected by Fluid-Attenuated Inversion Recovery MR Imaging in Patients with Spontaneous Intracranial Hypotension. American Journal of Neuroradiology 2008;29:1164–70. Doi:10.3174/ajnr.A1041. DOI: https://doi.org/10.3174/ajnr.A1041
24. Williams J, Brinjikji W, Cutsforth-Gregory JK. Natural history of spontaneous intracranial hypotension: a clinical and imaging study. J Neurointerv Surg 2023;15:1124–8. Doi:10.1136/jnis-2022-019300. DOI: https://doi.org/10.1136/jnis-2022-019300
25. Chen S-T, Wu J-W, Wang Y-F, Lirng J-F, Hseu S-S, Wang S-J. The time sequence of brain MRI findings in spontaneous intracranial hypotension. Cephalalgia 2022;42:12–9. Doi:10.1177/03331024211044424. DOI: https://doi.org/10.1177/03331024211044424
26. Callen AL, Timpone VM, Schwertner A, Zander D, Grassia F, Lennarson P, et al. Algorithmic Multimodality Approach to Diagnosis and Treatment of Spinal CSF Leak and Venous Fistula in Patients With Spontaneous Intracranial Hypotension. American Journal of Roentgenology 2022;219:292–301. Doi:10.2214/AJR.22.27485. DOI: https://doi.org/10.2214/AJR.22.27485
27. Lützen N, Beck J, Carlton Jones L, Fung C, Demerath T, Rau A, et al. MRI and Surgical Findings Refine Concepts of Type 2 Cerebrospinal Fluid Leaks in Spontaneous Intracranial Hypotension. Radiology 2025;314. Doi:10.1148/radiol.241653. DOI: https://doi.org/10.1148/radiol.241653
28. Carlton Jones L, Edelmuth DGL, Butteriss D, Scoffings DJ. “Flow Void Sign”: Flow Artifact on T2-Weighted MRI Can Be an Indicator of Dural Defect Location in Ventral Type 1 Spinal CSF Leaks. American Journal of Neuroradiology 2025;46:211–8. Doi:10.3174/ajnr.A8445. DOI: https://doi.org/10.3174/ajnr.A8445
29. Wegscheid ML, Chatterjee AR, Raji CA, Reis MN, Fleege NP, Azad SN, et al. Mesoscale CISS Imaging for the Detection of Dural Defects in Spinal CSF Leaks 2025. Doi:10.1101/2025.07.14.25331467. DOI: https://doi.org/10.1101/2025.07.14.25331467
30. Rajbhandari S, Petutschnigg T, Häni L, Nasiri D, Goldberg J, Schankin C, et al. Locating Spinal Leaks in Spontaneous Intracranial Hypotension: How Many Dynamic Myelographies Does It Take? American Journal of Neuroradiology 2025. Doi:10.3174/ajnr.A9032. DOI: https://doi.org/10.3174/ajnr.A9032
31. Tay AS-MS, Maya MM, Kranz PG, Madhavan AA, Schievink WI. Systematic Review of the Predictive Value of Negative Brain or Low Probability Brain MRIs in Patients with CSF-Venous Fistulas. American Journal of Neuroradiology 2025;46:2673–9. Doi:10.3174/ajnr.A8884. DOI: https://doi.org/10.3174/ajnr.A8884
32. Gibby JT, Amrhein TJ, Young DS, Houk JL, Kranz PG. Diagnostic Yield of Decubitus CT Myelography for Detection of CSF-Venous Fistulas. American Journal of Neuroradiology 2024;45:1597–604. Doi:10.3174/ajnr.A8330. DOI: https://doi.org/10.3174/ajnr.A8330
33. Schievink WI, Maya MM, Tay AS-MS, Nisson PL, Acharya J, Taché RB, et al. Optic Nerve Sheath MR Imaging Measurements in Patients with Orthostatic Headaches and Normal Findings on Conventional Imaging Predict the Presence of an Underlying CSF-Venous Fistula. American Journal of Neuroradiology 2024;45:655–61. Doi:10.3174/ajnr.A8165. DOI: https://doi.org/10.3174/ajnr.A8165
34. Cheema S, Anderson J, Angus-Leppan H, Armstrong P, Butteriss D, Carlton Jones L, et al. Multidisciplinary consensus guideline for the diagnosis and management of spontaneous intracranial hypotension. J Neurol Neurosurg Psychiatry 2023;94:835–43. Doi:10.1136/jnnp-2023-331166. DOI: https://doi.org/10.1136/jnnp-2023-331166
35. Schievink WI, Maya M, Prasad RS, Wadhwa VS, Cruz RB, Moser FG, et al. Spontaneous spinal cerebrospinal fluid‐venous fistulas in patients with orthostatic headaches and normal conventional brain and spine imaging. Headache: The Journal of Head and Face Pain 2021;61:387–91. Doi:10.1111/head.14048. DOI: https://doi.org/10.1111/head.14048
36. Kranz PG, Amrhein TJ, Gray L. CSF Venous Fistulas in Spontaneous Intracranial Hypotension: Imaging Characteristics on Dynamic and CT Myelography. American Journal of Roentgenology 2017;209:1360–6. Doi:10.2214/AJR.17.18351. DOI: https://doi.org/10.2214/AJR.17.18351
37. Orscelik A, Senol YC, Musmar B, Kobeissi H, Bilgin GB, Zandpazandi S, et al. Endovascular embolization of cerebrospinal fluid-venous fistula: a comprehensive systematic review on its efficacy and safety for the management of spontaneous intracranial hypotension. Neurosurg Rev 2024;47:28. Doi:10.1007/s10143-023-02264-1. DOI: https://doi.org/10.1007/s10143-023-02264-1
38. Wu J-W, Hseu S-S, Fuh J-L, Lirng J-F, Wang Y-F, Chen W-T, et al. Factors predicting response to the first epidural blood patch in spontaneous intracranial hypotension. Brain 2017;140:344–52. Doi:10.1093/brain/aww328. DOI: https://doi.org/10.1093/brain/aww328
39. Brinjikji W, Savastano LE, Atkinson JLD, Garza I, Farb R, Cutsforth-Gregory JK. A Novel Endovascular Therapy for CSF Hypotension Secondary to CSF-Venous Fistulas. American Journal of Neuroradiology 2021;42:882–7. Doi:10.3174/ajnr.A7014. DOI: https://doi.org/10.3174/ajnr.A7014
40. Gensler R, Balu A, Kraus A, Buck P, Bryant J-P, Cobourn K, et al. Surgical Treatment of Spontaneous Intracranial Hypotension From Spinal Cerebrospinal Fluid Leak: Single Institution Case Series. Neurosurgery 2025;97:1132–40. Doi:10.1227/neu.0000000000003543. DOI: https://doi.org/10.1227/neu.0000000000003543
41. Konovalov A, Gadzhiagaev V, Vinogradov E, Nikitin N, Eliava S, Konovalov N. Surgical Treatment Efficacy of CSF-Venous Fistulas: Systematic Review. World Neurosurg 2022;161:91–6. Doi:10.1016/j.wneu.2022.02.036. DOI: https://doi.org/10.1016/j.wneu.2022.02.036
42. Hong JH, Lee HW, Lee YH. Greater occipital nerve blockade using ultrasound guidance for the headache of spontaneous intracranial hypotension - A case report -. Anesth Pain Med (Seoul) 2022;17:62–6. Doi:10.17085/apm.21082. DOI: https://doi.org/10.17085/apm.21082
Downloads
Published
Issue
Section
License
Copyright (c) 2026 Marcio Nattan Portes Souza, Bernardo Uribe, Lucia Zavala, Reydmar Lopez, Vanesa Nagel, Marcelo Calderaro, Diogo Guilherme Edelmuth (Author)
This work is licensed under a Creative Commons Attribution 4.0 International License.
