Modern approaches to the diagnosis and treatment of spontaneous intracranial hypotension (a literature review)

Authors

DOI:

https://doi.org/10.14739/2310-1210.2022.2.235648

Keywords:

spontaneous intracranial hypotension, etiology, diagnosis, treatment

Abstract

The aim of this review is to analyze and summarize the existing evidence regarding the diagnosis and treatment of spontaneous intracranial hypotension.

Spontaneous intracranial hypotension occurs due to a decrease in cerebrospinal fluid (CSF) volume as a result of its spontaneous leakage and has a variety of clinical and neuroimaging features, sometimes different from CSF hypotension after a lumbar puncture. Anatomical changes in spontaneous CSF leakage are complex and are often associated with structural disorders of the connective tissue, weakness of certain areas of the dura mater and its diverticulosis. One consequence of decreased CSF volume is brain descent. This leads to traction of pain-sensitive brain structures and, therefore, to headaches that are orthostatic or with some orthostatic features. The methods of choice to identify the site of CSF leak are CT-myelography and radioisotope cisternography. Epidural blood patch is the main treatment in most cases.

Conclusions. The study on the most informative and minimally invasive methods to locate the site of CSF leak, conservative and surgical methods of treatment efficacy evaluation depending on the etiological factors of spontaneous intracranial hypotension are extremely important and promising scientific fields requiring further research with the development of personalized approaches considering the individual characteristics of each patient.

Author Biographies

K. A. Stepanchenko, Kharkiv Medical Academy of Postgraduate Education, Ukraine

MD, PhD, DSc, Professor of the Department of Neurology and Neurosurgery

V. H. Marchenko, Kharkiv Medical Academy of Postgraduate Education, Ukraine

MD, PhD, DSc, Professor of the Department of Neurology and Neurosurgery

Yu. V. Shmatko, Kharkiv Medical Academy of Postgraduate Education, Ukraine

MD, PhD, Associate Professor of the Department of Neurology and Neurosurgery

O. B. Bondar, Kharkiv Medical Academy of Postgraduate Education, Ukraine

MD, PhD, Assistant of the Department of Neurology and Neurosurgery

References

International Headache Society. (2018). Headache Classification Committee of the International Headache Society (IHS) The International Classification of Headache Disorders, 3rd edition. Cephalalgia, 38(1), 1-211. https://doi.org/10.1177/0333102417738202

Kranz, P. G., Gray, L., & Amrhein, T. J. (2018). Spontaneous Intracranial Hypotension: 10 Myths and Misperceptions. Headache, 58(7), 948-959. https://doi.org/10.1111/head.13328

Subramanian, A., Kecler-Pietrzyk, A., & Murphy, S. M. (2020). Spontaneous intracranial hypotension-a common misdiagnosis. QJM: An International Journal of Medicine, 113(6), 421-422. https://doi.org/10.1093/qjmed/hcaa012

Graff-Radford, S. B., & Schievink, W. I. (2014). High-Pressure Headaches, Low-Pressure Syndromes, and CSF Leaks: Diagnosis and Management. Headache, 54(2), 394-401. https://doi.org/10.1111/head.12283

Upadhyaya, P., & Ailani, J. (2019). A Review of Spontaneous Intracranial Hypotension. Current Neurology and Neuroscience Reports, 19(5), Article 22. https://doi.org/10.1007/s11910-019-0938-7

Perez-Vega, C., Robles-Lomelin, P., Robles-Lomelin, I., & Garcia Navarro, V. (2020). Spontaneous intracranial hypotension: key features for a frequently misdiagnosed disorder. Neurological Sciences, 41(9), 2433-2441. https://doi.org/10.1007/s10072-020-04368-8

Hébert-Blouin, M. N., Mokri, B., Shin, A. Y., Bishop, A. T., & Spinner, R. J. (2013). Cerebrospinal fluid volume-depletion headaches in patients with traumatic brachial plexus injury. Journal of Neurosurgery, 118(1), 149-154. https://doi.org/10.3171/2012.9.JNS112368

Cultrera, F., Lofrese, G., & Nasi, M. T. (2019). Spontaneous intracranial hypotension due to sacral diverticula: Two-case history and a pocket-sized review. Neurocirugía, 30(5), 228-232. https://doi.org/10.1016/j.neucir.2018.06.003

Cohen-Gadol, A. A., Mokri, B., Piepgras, D. G., Meyer, F. B., & Atkinson, J. L. D. (2006). Surgical Anatomy Of Dural Defects In Spontaneous Spinal Cerebrospinal Fluid Leaks. Operative Neurosurgery, 58(Suppl. 4), ONS-238-ONS-245. https://doi.org/10.1227/01.NEU.0000204712.16099.FB

Mokri, B., Maher, C. O., & Sencakova, D. (2002). Spontaneous CSF leaks: Underlying disorder of connective tissue. Neurology, 58(5), 814-816. https://doi.org/10.1212/wnl.58.5.814

Schrijver, I., Schievink, W. I., Godfrey, M., Meyer, F. B., & Francke, U. (2002). Spontaneous spinal cerebrospinal fluid leaks and minor skeletal features of Marfan syndrome: a microfibrillopathy. Journal of Neurosurgery, 96(3), 483-489. https://doi.org/10.3171/jns.2002.96.3.0483

Mokri, B. (2008). Familial Occurrence of Spontaneous Spinal CSF Leaks: Underlying Connective Tissue Disorder (CME). Headache, 48(1), 146-149. https://doi.org/10.1111/j.1526-4610.2007.00979.x

Eross, E. J., Dodick, D. W., Nelson, K. D., Bosch, P., & Lyons, M. (2002). Orthostatic Headache Syndrome with Csf Leak Secondary To Bony Pathology of the Cervical Spine. Cephalalgia, 22(6), 439-443. https://doi.org/10.1046/j.1468-2982.2002.00385.x

Winter, S. C., Maartens, N. F., Anslow, P., & Teddy, P. J. (2002). Spontaneous intracranial hypotension due to thoracic disc herniation. Case report. Journal of Neurosurgery: Spine, 96(3), 343-345. https://doi.org/10.3171/spi.2002.96.3.0343

Kranz, P. G., Gray, L., Malinzak, M. D., & Amrhein, T. J. (2019). Spontaneous Intracranial Hypotension: Pathogenesis, Diagnosis, and Treatment. Neuroimaging Clinics of North America, 29(4), 581-594. https://doi.org/10.1016/j.nic.2019.07.006

Sakano, H., Jafari, A., Allehaiby, W., & Harris, J. P. (2020). Spontaneous Intracranial Hypotension May Be an Under-recognized Cause of Endolymphatic Hydrops. Otology & Neurotology, 41(7), e860-e863. https://doi.org/10.1097/MAO.0000000000002665

Li, C., Raza, H. K., Chansysouphanthong, T., Zu, J., & Cui, G. (2019). A clinical analysis on 40 cases of spontaneous intracranial hypotension syndrome. Somatosensory & Motor Research, 36(1), 24-30. https://doi.org/10.1080/08990220.2019.1566122

Sugiura, M., Shibata, K., Saito, S., Nishimura, Y., Takahashi, K., & Sakura, H. (2019). A case of spontaneous intracranial hypotension with transient neurological symptoms. Rinsho Shinkeigaku, 59(8), 530-535. https://doi.org/10.5692/clinicalneurol.cn-001298

Mokri, B. (2013). Spontaneous Low Pressure, Low CSF Volume Headaches: Spontaneous CSF Leaks. Headache, 53(7), 1034-1053. https://doi.org/10.1111/head.12149

Niraj, G., Critchley, P., Kodivalasa, M., & Dorgham, M. (2017). Greater Occipital Nerve Treatment in the Management of Spontaneous Intracranial Hypotension Headache: A Case Report. Headache, 57(6), 952-955. https://doi.org/10.1111/head.13095

Mathew, P. G., & Cutrer, F. M. (2014). Injecting Under Pressure: The Pain of Low CSF Pressure Headache Responsive to Botulinum Toxin Injections. Current Neurology and Neuroscience Reports, 14(9), Article 477. https://doi.org/10.1007/s11910-014-0477-1

Mea, E., Chiapparini, L., Savoiardo, M., Franzini, A., Bussone, G., & Leone, M. (2008). Headache attributed to spontaneous intracranial hypotension. Neurological Sciences, 29(Suppl. 1), Article 164. https://doi.org/10.1007/s10072-008-0914-5

Stepanchenko, K. A. (2017). Myshechnaya disfunktsiya pri golovnoi boli napryazheniya u podrostkov i metody ee korrektsii [Muscle dysfunction in tension-type headache in adolescents and methods of its correction]. Medychni perspektyvy, 22(1), 76-80. https://doi.org/10.26641/2307-0404.2017.1.101420 [in Russian].

Primalani, N. K., Quek, T. J., Low, D., & Low, S. (2019). Spontaneous Intracranial Hypotension Presenting As Cervicogenic Headache: Case Report and Review of Literature. World Neurosurgery, 130, 550-554. https://doi.org/10.1016/j.wneu.2019.05.107

Mokri, B. (2002). Spontaneous Csf Leaks Mimicking Benign Exertional Headaches. Cephalalgia, 22(10), 780-783. https://doi.org/10.1046/j.1468-2982.2002.00400.x

Schievink, W. I., Wijdicks, E. F., Meyer, F. B., & Sonntag, V. K. (2001). Spontaneous Intracranial Hypotension Mimicking Aneurysmal Subarachnoid Hemorrhage. Neurosurgery, 48(3), 513-517. https://doi.org/10.1097/00006123-200103000-00009

Leep Hunderfund, A. N., & Mokri, B. (2012). Second-half-of-the-day headache as a manifestation of spontaneous CSF leak. Journal of Neurology, 259(2), 306-310. https://doi.org/10.1007/s00415-011-6181-z

Mokri, B., Aksamit, A. J., & Atkinson, J. L. (2004). Paradoxical Postural Headaches in Cerebrospinal Fluid Leaks. Cephalalgia, 24(10), 883-887. https://doi.org/10.1111/j.1468-2982.2004.00763.x

Pensato, U., Giammello, F., Baldini, T., Zaniboni, A., Piccolo, L., Arnone, G., Gentile, M., Cirillo, L., Simonetti, L., Isceri, S., & Zini, A. (2021). The domino effect of acephalgic spontaneous intracranial hypotension. Neurological Sciences, 42(1), 309-312. https://doi.org/10.1007/s10072-020-04755-1

Xia, P., Zhang, S. R., Zhou, Z. J., Shao, Y. Q., & Hu, X. Y. (2018). Benign paroxysmal positional vertigo in spontaneous intracranial hypotension. Neurological Research, 40(10), 868-873. https://doi.org/10.1080/01616412.2018.1495883

Hong, M., Shah, G. V., Adams, K. M., Turner, R. S., & Foster, N. L. (2002). Spontaneous intracranial hypotension causing reversible frontotemporal dementia. Neurology, 58(8), 1285-1287. https://doi.org/10.1212/wnl.58.8.1285

Mokri, B. (2014). Movement disorders associated with spontaneous CSF leaks: A case series. Cephalalgia, 34(14), 1134-1141. https://doi.org/10.1177/0333102414531154

Kranz, P. G., Malinzak, M. D., Amrhein, T. J., & Gray, L. (2017). Update on the Diagnosis and Treatment of Spontaneous Intracranial Hypotension. Current Pain and Headache Reports, 21(8), Article 37. https://doi.org/10.1007/s11916-017-0639-3

Neilson, D., & Martin, V. T. (2014). Joint Hypermobility and Headache: Understanding the Glue That Binds the Two Together - Part 1. Headache, 54(8), 1393-1402. https://doi.org/10.1111/head.12418

Rozen, T., Swidan, S., Hamel, R., & Saper, J. (2008). Trendelenburg Position: A Tool to Screen for the Presence of a Low CSF Pressure Syndrome in Daily Headache Patients. Headache, 48(9), 1366-1371. https://doi.org/10.1111/j.1526-4610.2007.01027.x

Schievink, W. I., Dodick, D. W., Mokri, B., Silberstein, S., Bousser, M. G., & Goadsby, P. J. (2011). Diagnostic Criteria for Headache Due to Spontaneous Intracranial Hypotension: A Perspective. Headache, 51(9), 1442-1444. https://doi.org/10.1111/j.1526-4610.2011.01911.x

Balkan, I. I., Albayram, S., Ozaras, R., Yilmaz, M. H., Ozbayrak, M., Mete, B., Yemisen, M., & Tabak, F. (2012). Spontaneous intracranial hypotension syndrome may mimic aseptic meningitis. Scandinavian Journal of Infectious Diseases, 44(7), 481-488. https://doi.org/10.3109/00365548.2012.664776

Mokri, B. (2014). Radioisotope Cisternography in Spontaneous CSF Leaks: Interpretations and Misinterpretations. Headache, 54(8), 1358-1368. https://doi.org/10.1111/head.12421

Farb, R. I., Nicholson, P. J., Peng, P. W., Massicotte, E. M., Lay, C., Krings, T., & terBrugge, K. G. (2019). Spontaneous Intracranial Hypotension: A Systematic Imaging Approach for CSF Leak Localization and Management Based on MRI and Digital Subtraction Myelography. American Journal of Neuroradiology, 40(4), 745-753. https://doi.org/10.3174/ajnr.A6016

Bond, K. M., Benson, J. C., Cutsforth-Gregory, J. K., Kim, D. K., Diehn, F. E., & Carr, C. M. (2020). Spontaneous Intracranial Hypotension: Atypical Radiologic Appearances, Imaging Mimickers, and Clinical Look-Alikes. American Journal of Neuroradiology, 41(8), 1339-1347. https://doi.org/10.3174/ajnr.A6637

Kinsman, K. A., Verdoorn, J. T., Luetmer, P. H., Clark, M. S., & Diehn, F. E. (2019). Renal Contrast on CT Myelography: Diagnostic Value in Patients with Spontaneous Intracranial Hypotension. American Journal of Neuroradiology, 40(2), 376-381. https://doi.org/10.3174/ajnr.A5934

Yoon, M. K., Parsa, A. T., & Horton, J. C. (2013). Skull thickening, paranasal sinus expansion, and sella turcica shrinkage from chronic intracranial hypotension. Journal of Neurosurgery: Pediatrics, 11(6), 667-672. https://doi.org/10.3171/2013.2.PEDS12560

Amrhein, T. J., & Kranz, P. G. (2019). Spontaneous Intracranial Hypotension: Imaging in Diagnosis and Treatment. Radiologic Clinics of North America, 57(2), 439-451. https://doi.org/10.1016/j.rcl.2018.10.004

Shah, V. N., & Dillon, W. P. (2020). Spontaneous Intracranial Hypotension With Brain Sagging Attributable to a Cerebrospinal Fluid-Venous Fistula. JAMA Neurology, 77(10), 1320-1321. https://doi.org/10.1001/jamaneurol.2020.2372

Wu, J. W., Wang, Y. F., Fuh, J. L., Lirng, J. F., Chen, S. P., Hseu, S. S., & Wang, S. J. (2018). Correlations among brain and spinal MRI findings in spontaneous intracranial hypotension. Cephalalgia, 38(14), 1998-2005. https://doi.org/10.1177/0333102418804161

Beck, J., Ulrich, C. T., Fung, C., Fichtner, J., Seidel, K., Fiechter, M., Hsieh, K., Murek, M., Bervini, D., Meier, N., Mono, M. L., Mordasini, P., Hewer, E., Z'Graggen, W. J., Gralla, J., & Raabe, A. (2016). Diskogenic microspurs as a major cause of intractable spontaneous intracranial hypotension. Neurology, 87(12), 1220-1226. https://doi.org/10.1212/WNL.0000000000003122

Mokri, B. (2001). The Monro-Kellie hypothesis: Applications in CSF volume depletion. Neurology, 56(12), 1746-1748. https://doi.org/10.1212/wnl.56.12.1746

Spelle, L., Boulin, A., Tainturier, C., Visot, A., Graveleau, P., & Pierot, L. (2001). Neuroimaging features of spontaneous intracranial hypotension. Neuroradiology, 43(8), 622-627. https://doi.org/10.1007/s002340000529

Pensato, U., Russo, C., Favoni, V., Cirillo, L., Asioli, G. M., Cortelli, P., Cevoli, S., & Pierangeli, G. (2020). Clinical and radiological prognostic factors in spontaneous intracranial hypotension: a case series. Neurological Sciences, 41(Suppl. 2), 475-477. https://doi.org/10.1007/s10072-020-04664-3

Luetmer, P. H., & Mokri, B. (2003). Dynamic CT Myelography: A Technique for Localizing High-Flow Spinal Cerebrospinal Fluid Leaks. American Journal of Neuroradiology, 24(8), 1711-1714.

Akbar, J. J., Luetmer, P. H., Schwartz, K. M., Hunt, C. H., Diehn, F. E., & Eckel, L. J. (2012). The Role of MR Myelography with Intrathecal Gadolinium in Localization of Spinal CSF Leaks in Patients with Spontaneous Intracranial Hypotension. American Journal of Neuroradiology, 33(3), 535-540. https://doi.org/10.3174/ajnr.A2815

Davidson, B., Nassiri, F., Mansouri, A., Badhiwala, J. H., Witiw, C. D., Shamji, M. F., Peng, P. W., Farb, R. I., & Bernstein, M. (2017). Spontaneous Intracranial Hypotension: A Review and Introduction of an Algorithm For Management. World Neurosurgery, 101, 343-349. https://doi.org/10.1016/j.wneu.2017.01.123

Starling, A., Hernandez, F., Hoxworth, J. M., Trentman, T., Halker, R., Vargas, B. B., Hastriter, E., & Dodick, D. (2013). Sensitivity of MRI of the spine compared with CT myelography in orthostatic headache with CSF leak. Neurology, 81(20), 1789-1792. https://doi.org/10.1212/01.wnl.0000435555.13695.22

Zhang, D., Wang, J., Zhang, Q., He, F., & Hu, X. (2018). Cerebral Venous Thrombosis in Spontaneous Intracranial Hypotension: A Report on 4 Cases and a Review of the Literature. Headache, 58(8), 1244-1255. https://doi.org/10.1111/head.13413

D'Amico, D., Usai, S., Chiapparini, L., Erbetta, A., Gioppo, A., Messina, G., Astengo, A., & Leone, M. (2020). Headache in spontaneous intracranial hypotension: an overview with indications for differential diagnosis in the clinical practice. Neurological Sciences, 41(Suppl. 2), 423-427. https://doi.org/10.1007/s10072-020-04642-9

Leep Hunderfund, A. N., & Mokri, B. (2008). Orthostatic headache without CSF leak. Neurology, 71(23), 1902-1906. https://doi.org/10.1212/01.wnl.0000336655.07617.e0

Olesen, J., Tfelt-Hansen, P., & Welch, K. M. A. (Eds.). (2000). The Headaches (2nd ed.). Lippincott, Williams & Wilkins.

Mokri, B. (2014). Spontaneous CSF Leaks: Low CSF Volume Syndromes. Neurologic Clinics, 32(2), 397-422. https://doi.org/10.1016/j.ncl.2013.11.002

Gentile, S., Giudice, R. L., Martino, P. D., Rainero, I., & Pinessi, L. (2004). Headache attributed to spontaneous low CSF pressure: report of three cases responsive to corticosteroids. European Journal of Neurology, 11(12), 849-851. https://doi.org/10.1111/j.1468-1331.2004.00898.x

Binder, D. K., Dillon, W. P., Fishman, R. A., & Schmidt, M. H. (2002). Intrathecal saline infusion in the treatment of obtundation associated with spontaneous intracranial hypotension: technical case report. Neurosurgery, 51(3), 830-837.

Uyar Türkyilmaz, E., Camgöz Eryilmaz, N., Aydin Güzey, N., & Moraloğlu, Ö. (2016). Bilateral greater occipital nerve block for treatment of post-dural puncture headache after caesarean operations. Brazilian Journal of Anesthesiology, 66(5), 445-450. https://doi.org/10.1016/j.bjane.2015.03.004

Rajesh, M. C., Noone, M. L., & Harish Babu, P. S. (2019). Epidural blood patch for spontaneous intracranial hypotension. The National Medical Journal of India, 32(5), 288-289. https://doi.org/10.4103/0970-258X.295965

Sencakova, D., Mokri, B., & McClelland, R. L. (2001). The efficacy of epidural blood patch in spontaneous CSF leaks. Neurology, 57(10), 1921-1923. https://doi.org/10.1212/wnl.57.10.1921

Crul, B. J., Gerritse, B. M., van Dongen, R. T., & Schoonderwaldt, H. C. (1999). Epidural Fibrin Glue Injection Stops Persistent Postdural Puncture Headache. Anesthesiology, 91(2), 576-577. https://doi.org/10.1097/00000542-199908000-00039

Arumugam, G., Ram, S., Bhaskar Naidu P., & Kumaravelu, S. (2020). Epidural blood patch for spontaneous intracranial hypotension (SIH): a report of two cases. Tropical Doctor, 50(4), 369-373. https://doi.org/10.1177/0049475520933140

Ferrante, E., Arpino, I., Citterio, A., Wetzl, R., & Savino, A. (2010). Epidural blood patch in Trendelenburg position pre-medicated with acetazolamide to treat spontaneous intracranial hypotension. European Journal of Neurology, 17(5), 715-719. https://doi.org/10.1111/j.1468-1331.2009.02913.x

Akbar, R. A., Khan, A. A., Fernandes, G. M., Ahmed Mohamed, A. Z., Elsotouhy, A., & Ali, Y. (2020). Spontaneous Intracranial Hypotension and Its Management with a Cervical Epidural Blood Patch: A Case Report. The American Journal of Case Reports, 21, Article e925986. https://doi.org/10.12659/AJCR.925986

Santos, J. P., Silva, A. M., Velasco, R. M., & Freitas, M. J. (2020). Lumbar epidural blood patch for the treatment of spontaneous intracranial hypotension. Journal of Clinical Anesthesia, 60, Article 11. https://doi.org/10.1016/j.jclinane.2019.08.014

Schievink, W. I., Morreale, V. M., Atkinson, J. L., Meyer, F. B., Piepgras, D. G., & Ebersold, M. J. (1998). Surgical treatment of spontaneous spinal cerebrospinal fluid leaks. Journal of Neurosurgery, 88(2), 243-246. https://doi.org/10.3171/jns.1998.88.2.0243

Schievink, W. I. (2006). Spontaneous Spinal Cerebrospinal Fluid Leaks and Intracranial Hypotension. JAMA, 295(19), 2286-2296. https://doi.org/10.1001/jama.295.19.2286

Mostofi, E., Schievink, W. I., & Sim, V. L. (2016). Dural Reduction Surgery: A Treatment Option for Frontotemporal Brain Sagging Syndrome. Canadian Journal of Neurological Sciences, 43(4), 593-595. https://doi.org/10.1017/cjn.2016.3

Published

2022-04-04

How to Cite

1.
Stepanchenko KA, Marchenko VH, Shmatko YV, Bondar OB. Modern approaches to the diagnosis and treatment of spontaneous intracranial hypotension (a literature review). Zaporozhye medical journal [Internet]. 2022Apr.4 [cited 2024Apr.19];24(2):230-9. Available from: http://zmj.zsmu.edu.ua/article/view/235648

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