Cerebral Venous Thrombosis as a Complication of Spontaneous Intracranial Hypotension: A Case Report
-
Mohammad Ali Nahayati
,
Maryam Payere
,
Amir Khanmirzaei
,
Bahar Karimikhoshnoudian
,
Soheil Shokri-shakib
Abstract
Spontaneous intracranial hypotension (SIH) is an uncommon condition caused by cerebrospinal fluid (CSF) leakage, which can lead to complications such as cerebral venous thrombosis (CVT), occurring in 1–2% of SIH patients. This case report describes the presentation, diagnosis, and management of SIH complicated by CVT. A 36-year-old woman with no comorbidities presented with a three-week history of severe, initially orthostatic headaches. Neurological examination revealed mild bilateral disc oedema, and imaging showed generalized brain oedema. MRI with MRV confirmed cerebral venous sinus thrombosis along with typical SIH features, including pachymeningeal enhancement and venous engorgement. She was treated with heparin infusion and
isotonic saline, responding well without seizures or hemorrhagic complications. This case highlights the risk of CVT in SIH and underscores the importance of early diagnosis through neuroimaging and lumbar puncture. The patient’s favorable response to anticoagulation demonstrates effective management, though further
studies are needed to establish definitive treatment guidelines for SIH-associated CVT.
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org/10.1212/CON.0000000000000193
[2] Arnold M. Headache classification committee of the international headache society (IHS): the international
classification of headache disorders. Cephalalgia. 2018;38(1):1- 211. https://doi.org/10.1177/0333102417738202
[3] Schievink WI. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. JAMA. 2006;295(19):2286-96. https://doi.org/10.1001/jama.295.19.2286
[4] Schwedt TJ, Dodick DW. Spontaneous intracranial hypotension. Curr Pain Headache Rep. 2007;11:56-61. https://
doi.org/10.1007/s11916-007-0023-9
[5] Ferrante E, Trimboli M, Petrecca G, Allegrini F, Ferrante MM, Rubino F. Management of spontaneous intracranial
hypotension during pregnancy: a case series. Headache. 2020;60(8):1777-87. https://doi.org/10.1111/head.13942
[6] Headache Classification Committee of the International Headache Society (IHS). The international classification of
headache disorders, 3rd edition. Cephalalgia. 2018;38(1):1- 211. https://doi.org/10.1177/0333102417738202
[7] Ferrante E, Pontrelli G, Rubino F, Trimboli M. Spontaneous intracranial hypotension with brain sagging causing “prayer headache”. Rev Neurol (Paris). 2021;177(3):321-3. https://doi. org/10.1016/j.neurol.2020.06.003
[8] Schievink WI. Spontaneous intracranial hypotension. N Engl J Med. 2021;385(23):2173-8. https://doi.org/10.1056/
NEJMra2101561
[9] Ferrante E, Olgiati E, Sangalli V, Rubino F. Early pain relief from orthostatic headache and hearing changes in spontaneousintracranial hypotension after epidural blood patch. Acta Neurol Belg. 2016;116(4):503-8. https://doi.org/10.1007/s13760-016-0617-2
[10] Friedman DI. Headaches due to low and high intracranial pressure. Continuum (Minneap Minn). 2018;24(4):1066-91.
https://doi.org/10.1212/CON.0000000000000623
[11] Wiesemann E, Berding G, Goetz F, Windhagen A. Spontaneous intracranial hypotension: correlation of imaging findings with clinical features. Eur Neurol. 2006;56(4):204-10. https://doi.org/10.1159/000096487
[12] Schievink WI. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. JAMA. 2006;295(19):2286-96. https://doi.org/10.1001/jama.295.19.2286
[13] Mokri B, Piepgras DG, Miller GM. Syndrome of orthostatic headaches and diffuse pachymeningeal gadolinium
enhancement. Mayo Clin Proc. 1997;72(5):400-13. https://doi.org/10.4065/72.5.400
[14] Savoiardo M, Armenise S, Spagnolo P, De Simone T, Mandelli ML, Marcone A, et al. Dural sinus thrombosis in spontaneous intracranial hypotension: hypotheses on possible mechanisms. J Neurol. 2006;253(9):1197-202. https://doi.org/10.1007/s00415-006-0194-z
[15] Schievink WI, Maya MM. Cerebral venous thrombosis in spontaneous intracranial hypotension. Headache. 2008;48(10):1511-9. https://doi.org/10.1111/j.1526-4610.2008.01251.x
[16] Bousser MG, Ferro JM. Cerebral venous thrombosis: an update. Lancet Neurol. 2007;6(2):162-70. https://doi.
org/10.1016/S1474-4422(07)70029-7
[17] Mokri B. The Monro-Kellie hypothesis: applications in CSF volume depletion. Neurology. 2001;56(12):1746-8. https://doi.org/10.1212/WNL.56.12.1746
[18] Sopelana D, Marcos A, Arroyo R, Gutiérrez E, Cuenca R, Vázquez AV, et al. May intracranial hypotension be a cause
of venous sinus thrombosis? Eur Neurol. 2004;51(2):113-5. https://doi.org/10.1159/000076791
[19] Haritanti A, Karacostas D, Drevelengas A, Kanellopoulos V, Paraskevopoulou E, Lefkopoulos A, et al. Spontaneous
intracranial hypotension: clinical and neuroimaging findings in six cases with literature review. Eur J Radiol. 2009;69(2):253-9. https://doi.org/10.1016/j.ejrad.2007.10.013
[20] Park JH, Yoon SH. New concept of cerebrospinal fluid dynamics in cerebral venous sinus thrombosis. Med
Hypotheses. 2008;70(1):143-7. https://doi.org/10.1016/j. mehy.2007.03.036
[21] Ferro JM, Bousser MG, Canhão P, Coutinho JM, Crassard I, Dentali F, et al. European Stroke Organization guideline
for the diagnosis and treatment of cerebral venous thrombosis-endorsed by the European Academy of Neurology. Eur Stroke J. 2017;2(3):195-221. https://doi.org/10.1177/2396987317719364
[22] Risi G, Ducros A, van Dokkum L, Lonjon N, Marchi N, Villain M, et al. Prevalence, clinical presentation, and treatmentmanagement of cerebral venous thrombosis associated with spontaneous intracranial hypotension: a combined case-series and systematic literature review approach. Interv Neuroradiol. 2024:15910199241263139. https://doi.org/10.1177/15910199241263139
[23] Coutinho JM, Zuurbier SM, Bousser MG, Ji X, Canhão P, Roos YB, et al. Effect of endovascular treatment with
medical management vs standard care on severe cerebral venous thrombosis: the TO-ACT randomized clinical trial.
JAMA Neurol. 2020;77(8):966-73. https://doi.org/10.1001/jamaneurol.2020.1022
[24] Tang SZ, Jing M, Yang C, Yeo LLL, Tan BY, Chan BPL, et al. Safety and clinical outcomes in endovascular treatment
for symptomatic cerebral venous thrombosis: a singlecenter experience with meta-analysis. Neurosurg Rev.
2023;46(1):114. https://doi.org/10.1007/s10143-023-02012-5
[25] Lai TH, Fuh JL, Lirng JF, Tsai PH, Wang SJ. Subdural haematoma in patients with spontaneous intracranial hypotension. Cephalalgia. 2007;27(2):133-8. https://doi.org/10.1111/j.1468-2982.2006.01249.x
[26] Nardone R, Caleri F, Golaszewski S, Ladurner G, Tezzon F, Bailey A, et al. Subdural hematoma in a patient with spontaneous intracranial hypotension and cerebral venous thrombosis. Neurol Sci. 2010;31(5):669-72. https://doi.org/10.1007/s10072-010-0389-z
[27] Rice CM, Renowden SA, Sandeman DR, Cottrell DA. Spontaneous intracranial hypotension and venous sinus
thrombosis. Pract Neurol. 2013;13(2):120-4. https://doi.org/10.1136/practneurol-2012-00025774 Nahayati MA et al. Cerebral Venous Thrombosis Secondary to Spontaneous Intracranial Hypotension. CRCP. 2025; 10(2):69-74 March/April 2025, Volume 10, Issue 2
[28] Costa P, Del Zotto E, Giossi A, Volonghi I, Poli L, Frigerio M, et al. Headache due to spontaneous intracranial hypotension and subsequent cerebral vein thrombosis. Headache. 2012;52(10):1592-6. https://doi.org/10.1111/j.1526-4610.2012.02 261.x
[29] Dangra VR, Sharma YB, Bharucsha NE, Deopujari CE. An interesting case of headache. Ann Indian Acad Neurol.
2011;14(2):130-2. https://doi.org/10.4103/0972-2327. 82807
[30] Mao YT, Dong Q, Fu JH. Delayed subdural hematoma and cerebral venous thrombosis in a patient with spontaneous intracranial hypotension. Neurol Sci. 2011;32(5):981-3. https://doi.org/10.1007/s10072-011-0715-0
[31] Fujii N, Fujii H, Fujita A, Kim Y, Sugimoto H. Spontaneous intracranial hypotension complicated by cerebral venous
thrombosis. Radiol Case Rep. 2018;13(4):834-8. https://doi.org/10.1016/j.radcr.2018.05.014
[32] Rozen TD. Pachymeningeal enhancement on MRI: a venous phenomena not always related to intracranial hypotension (resolving pachymeningeal enhancement and cerebral vein thrombosis). Headache. 2013;53(4):673-5. https://doi.org/10.1111/head.12003
[33] Lan MY, Chang YY, Liu JS. Delayed cerebral venous thrombosis in a patient with spontaneous intracranial hypotension. Cephalalgia. 2007;27(10):1176-8. https://doi.org/10.1111/j.1468-2982.2007.01416.x
org/10.1212/CON.0000000000000193
[2] Arnold M. Headache classification committee of the international headache society (IHS): the international
classification of headache disorders. Cephalalgia. 2018;38(1):1- 211. https://doi.org/10.1177/0333102417738202
[3] Schievink WI. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. JAMA. 2006;295(19):2286-96. https://doi.org/10.1001/jama.295.19.2286
[4] Schwedt TJ, Dodick DW. Spontaneous intracranial hypotension. Curr Pain Headache Rep. 2007;11:56-61. https://
doi.org/10.1007/s11916-007-0023-9
[5] Ferrante E, Trimboli M, Petrecca G, Allegrini F, Ferrante MM, Rubino F. Management of spontaneous intracranial
hypotension during pregnancy: a case series. Headache. 2020;60(8):1777-87. https://doi.org/10.1111/head.13942
[6] Headache Classification Committee of the International Headache Society (IHS). The international classification of
headache disorders, 3rd edition. Cephalalgia. 2018;38(1):1- 211. https://doi.org/10.1177/0333102417738202
[7] Ferrante E, Pontrelli G, Rubino F, Trimboli M. Spontaneous intracranial hypotension with brain sagging causing “prayer headache”. Rev Neurol (Paris). 2021;177(3):321-3. https://doi. org/10.1016/j.neurol.2020.06.003
[8] Schievink WI. Spontaneous intracranial hypotension. N Engl J Med. 2021;385(23):2173-8. https://doi.org/10.1056/
NEJMra2101561
[9] Ferrante E, Olgiati E, Sangalli V, Rubino F. Early pain relief from orthostatic headache and hearing changes in spontaneousintracranial hypotension after epidural blood patch. Acta Neurol Belg. 2016;116(4):503-8. https://doi.org/10.1007/s13760-016-0617-2
[10] Friedman DI. Headaches due to low and high intracranial pressure. Continuum (Minneap Minn). 2018;24(4):1066-91.
https://doi.org/10.1212/CON.0000000000000623
[11] Wiesemann E, Berding G, Goetz F, Windhagen A. Spontaneous intracranial hypotension: correlation of imaging findings with clinical features. Eur Neurol. 2006;56(4):204-10. https://doi.org/10.1159/000096487
[12] Schievink WI. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. JAMA. 2006;295(19):2286-96. https://doi.org/10.1001/jama.295.19.2286
[13] Mokri B, Piepgras DG, Miller GM. Syndrome of orthostatic headaches and diffuse pachymeningeal gadolinium
enhancement. Mayo Clin Proc. 1997;72(5):400-13. https://doi.org/10.4065/72.5.400
[14] Savoiardo M, Armenise S, Spagnolo P, De Simone T, Mandelli ML, Marcone A, et al. Dural sinus thrombosis in spontaneous intracranial hypotension: hypotheses on possible mechanisms. J Neurol. 2006;253(9):1197-202. https://doi.org/10.1007/s00415-006-0194-z
[15] Schievink WI, Maya MM. Cerebral venous thrombosis in spontaneous intracranial hypotension. Headache. 2008;48(10):1511-9. https://doi.org/10.1111/j.1526-4610.2008.01251.x
[16] Bousser MG, Ferro JM. Cerebral venous thrombosis: an update. Lancet Neurol. 2007;6(2):162-70. https://doi.
org/10.1016/S1474-4422(07)70029-7
[17] Mokri B. The Monro-Kellie hypothesis: applications in CSF volume depletion. Neurology. 2001;56(12):1746-8. https://doi.org/10.1212/WNL.56.12.1746
[18] Sopelana D, Marcos A, Arroyo R, Gutiérrez E, Cuenca R, Vázquez AV, et al. May intracranial hypotension be a cause
of venous sinus thrombosis? Eur Neurol. 2004;51(2):113-5. https://doi.org/10.1159/000076791
[19] Haritanti A, Karacostas D, Drevelengas A, Kanellopoulos V, Paraskevopoulou E, Lefkopoulos A, et al. Spontaneous
intracranial hypotension: clinical and neuroimaging findings in six cases with literature review. Eur J Radiol. 2009;69(2):253-9. https://doi.org/10.1016/j.ejrad.2007.10.013
[20] Park JH, Yoon SH. New concept of cerebrospinal fluid dynamics in cerebral venous sinus thrombosis. Med
Hypotheses. 2008;70(1):143-7. https://doi.org/10.1016/j. mehy.2007.03.036
[21] Ferro JM, Bousser MG, Canhão P, Coutinho JM, Crassard I, Dentali F, et al. European Stroke Organization guideline
for the diagnosis and treatment of cerebral venous thrombosis-endorsed by the European Academy of Neurology. Eur Stroke J. 2017;2(3):195-221. https://doi.org/10.1177/2396987317719364
[22] Risi G, Ducros A, van Dokkum L, Lonjon N, Marchi N, Villain M, et al. Prevalence, clinical presentation, and treatmentmanagement of cerebral venous thrombosis associated with spontaneous intracranial hypotension: a combined case-series and systematic literature review approach. Interv Neuroradiol. 2024:15910199241263139. https://doi.org/10.1177/15910199241263139
[23] Coutinho JM, Zuurbier SM, Bousser MG, Ji X, Canhão P, Roos YB, et al. Effect of endovascular treatment with
medical management vs standard care on severe cerebral venous thrombosis: the TO-ACT randomized clinical trial.
JAMA Neurol. 2020;77(8):966-73. https://doi.org/10.1001/jamaneurol.2020.1022
[24] Tang SZ, Jing M, Yang C, Yeo LLL, Tan BY, Chan BPL, et al. Safety and clinical outcomes in endovascular treatment
for symptomatic cerebral venous thrombosis: a singlecenter experience with meta-analysis. Neurosurg Rev.
2023;46(1):114. https://doi.org/10.1007/s10143-023-02012-5
[25] Lai TH, Fuh JL, Lirng JF, Tsai PH, Wang SJ. Subdural haematoma in patients with spontaneous intracranial hypotension. Cephalalgia. 2007;27(2):133-8. https://doi.org/10.1111/j.1468-2982.2006.01249.x
[26] Nardone R, Caleri F, Golaszewski S, Ladurner G, Tezzon F, Bailey A, et al. Subdural hematoma in a patient with spontaneous intracranial hypotension and cerebral venous thrombosis. Neurol Sci. 2010;31(5):669-72. https://doi.org/10.1007/s10072-010-0389-z
[27] Rice CM, Renowden SA, Sandeman DR, Cottrell DA. Spontaneous intracranial hypotension and venous sinus
thrombosis. Pract Neurol. 2013;13(2):120-4. https://doi.org/10.1136/practneurol-2012-00025774 Nahayati MA et al. Cerebral Venous Thrombosis Secondary to Spontaneous Intracranial Hypotension. CRCP. 2025; 10(2):69-74 March/April 2025, Volume 10, Issue 2
[28] Costa P, Del Zotto E, Giossi A, Volonghi I, Poli L, Frigerio M, et al. Headache due to spontaneous intracranial hypotension and subsequent cerebral vein thrombosis. Headache. 2012;52(10):1592-6. https://doi.org/10.1111/j.1526-4610.2012.02 261.x
[29] Dangra VR, Sharma YB, Bharucsha NE, Deopujari CE. An interesting case of headache. Ann Indian Acad Neurol.
2011;14(2):130-2. https://doi.org/10.4103/0972-2327. 82807
[30] Mao YT, Dong Q, Fu JH. Delayed subdural hematoma and cerebral venous thrombosis in a patient with spontaneous intracranial hypotension. Neurol Sci. 2011;32(5):981-3. https://doi.org/10.1007/s10072-011-0715-0
[31] Fujii N, Fujii H, Fujita A, Kim Y, Sugimoto H. Spontaneous intracranial hypotension complicated by cerebral venous
thrombosis. Radiol Case Rep. 2018;13(4):834-8. https://doi.org/10.1016/j.radcr.2018.05.014
[32] Rozen TD. Pachymeningeal enhancement on MRI: a venous phenomena not always related to intracranial hypotension (resolving pachymeningeal enhancement and cerebral vein thrombosis). Headache. 2013;53(4):673-5. https://doi.org/10.1111/head.12003
[33] Lan MY, Chang YY, Liu JS. Delayed cerebral venous thrombosis in a patient with spontaneous intracranial hypotension. Cephalalgia. 2007;27(10):1176-8. https://doi.org/10.1111/j.1468-2982.2007.01416.x
| Files | ||
| Issue | Vol 10 No 2 (2025): March-April | |
| Section | Case Report(s) | |
| DOI | https://doi.org/10.18502/crcp.v10i2.19900 | |
| Keywords | ||
| Cerebral venous thrombosis Spontaneous intracranial hypotension Anticoagulation therapy Headache Neuroimaging | ||
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How to Cite
1.
Nahayati MA, Payere M, Khanmirzaei A, Karimikhoshnoudian B, Shokri-shakib S. Cerebral Venous Thrombosis as a Complication of Spontaneous Intracranial Hypotension: A Case Report. CRCP. 2025;10(2):69-74.
