Cerebral venous sinus thrombosis (CVST) has a range of underlying cause. Here a case of CVST with an uncommon etiology is presented and discussed. A 40-year-old female presented with headache for 2 years and progressive visual loss for 4 months. She had conjunctival congestion and bilateral papilloedema with pale disc on right side. Computed tomography (CT) scans and contrast magnetic resonance imaging (MRI) of brain were normal but venography (MRV) revealed widespread thrombosis of dural sinuses including superior sagittal sinus. Cerebrospianal fluid (CSF) pressure was high (360 mm of H2O) while other CSF parameters were normal. She was polycythemic (hemoglobin 18.1 g/dl, hematocrit 60.2%), Bone marrow study revealed pan-myeloid hyperplasia whereas trephine biopsy revealed hypercellular marrow with trilineage expansion suggestive of myeloproliferative disorder possibly polycythemia vera. JAK-2 mutation was also detected. CVST has different patterns of presentations including isolated intracranial hypertension leading to visual loss. It is essential to identify and treat underlying condition like polycythemia vera.
| Published in | Clinical Neurology and Neuroscience (Volume 5, Issue 3) |
| DOI | 10.11648/j.cnn.20210503.15 |
| Page(s) | 60-62 |
| Creative Commons |
This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited. |
| Copyright |
Copyright © The Author(s), 2024. Published by Science Publishing Group |
Cerebral Venous Sinus Thrombosis, Polycythemia Vera, Intracranial Hypertension
| [1] | Ferro JM, Bousser MG, Canhão P, Coutinho JM, Crassard I, Dentali F, di Minno M, Maino A, Martinelli I, Masuhr F, de Sousa DA. European Stroke Organization guideline for the diagnosis and treatment of cerebral venous thrombosis–endorsed by the European Academy of Neurology. European stroke journal. 2017 Sep; 2 (3): 195-221. |
| [2] | Coutinho JM, Zuurbier SM, Aramideh M, Stam J. The incidence of cerebral venous thrombosis: a cross-sectional study. Stroke. 2012 Dec; 43 (12): 3375-7. |
| [3] | Silvis SM, de Sousa DA, Ferro JM, Coutinho JM. Cerebral venous thrombosis. Nat Rev Neurol. 2017 Sep; 13 (9): 555-565. |
| [4] | Singh RJ, Saini J, Varadharajan S, Kulkarni GB, Veerendrakumar M. Headache in cerebral venous sinus thrombosis revisited: Exploring the role of vascular congestion and cortical vein thrombosis. Cephalalgia. 2018 Mar; 38 (3): 503-510. |
| [5] | Mehvari Habibabadi J, Saadatnia M, Tabrizi N. Seizure in cerebral venous and sinus thrombosis. Epilepsia Open. 2018 Jun 8; 3 (3): 316-322. |
| [6] | Rajoor UG, Sindhur JC, Gundikeri SK, Patil P. Cerebral Venous Thrombosis in Adults: An Experience from a Tertiary Teaching Hospital in Dharwad, Karnataka. Indian Journal of Public Health Research & Development. 2014 Jan 1; 5 (1). |
| [7] | Liu KC, Bhatti MT, Chen JJ, Fairbanks AM, Foroozan R, McClelland CM, Lee MS, Satija CE, Francis CE, Wildes MT, Subramanian PS, Williams ZR, El-Dairi MA. Presentation and Progression of Papilledema in Cerebral Venous Sinus Thrombosis. Am J Ophthalmol. 2020 May; 213: 1-8. |
| [8] | Leach JL, Fortuna RB, Jones BV, Gaskill-Shipley MF. Imaging of cerebral venous thrombosis: current techniques, spectrum of findings, and diagnostic pitfalls. Radiographics. 2006 Oct; 26 Suppl 1: S19-41; discussion S42-3. |
| [9] | Qu H, Yang M. Early imaging characteristics of 62 cases of cerebral venous sinus thrombosis. Exp Ther Med. 2013 Jan; 5 (1): 233-236. |
| [10] | Gao L, Xu W, Li T, Yu X, Cao S, Xu H, Yan F, Chen G. Accuracy of magnetic resonance venography in diagnosing cerebral venous sinus thrombosis. Thromb Res. 2018 Jul; 167: 64-73. |
| [11] | Pallewatte, A. S., Tharmalingam, T. and Liyanage, N., 2016. Anatomic variants and artefacts in non enhanced MRV – potential pitfalls in diagnosing cerebral venous sinus thrombosis (CVST). Sri Lanka Journal of Radiology, 2 (1), pp. 40–46. |
| [12] | Saposnik G, Barinagarrementeria F, Brown RD Jr, Bushnell CD, Cucchiara B, Cushman M, deVeber G, Ferro JM, Tsai FY; American Heart Association Stroke Council and the Council on Epidemiology and Prevention. Diagnosis and management of cerebral venous thrombosis: a statement for healthcare professionals from the American Heart Association/American Stroke Association. Stroke. 2011 Apr; 42 (4): 1158-92. |
| [13] | Stam J. Thrombosis of the cerebral veins and sinuses. N Engl J Med. 2005 Apr 28; 352 (17): 1791-8. |
| [14] | Tefferi A, Barbui T. Polycythemia vera and essential thrombocythemia: 2021 update on diagnosis, risk-stratification and management. Am J Hematol. 2020 Dec; 95 (12): 1599-1613. |
| [15] | Khan MWA, Zeeshan HM, Iqbal S. Clinical Profile and Prognosis of Cerebral Venous Sinus Thrombosis. Cureus. 2020 Dec 22; 12 (12): e12221. |
| [16] | Canhão P, Ferro JM, Lindgren AG, Bousser MG, Stam J, Barinagarrementeria F; ISCVT Investigators. Causes and predictors of death in cerebral venous thrombosis. Stroke. 2005 Aug; 36 (8): 1720-5. |
| [17] | Canhão P, Cortesão A, Cabral M, Ferro JM, Stam J, Bousser MG, Barinagarrementeria F; ISCVT Investigators. Are steroids useful to treat cerebral venous thrombosis? Stroke. 2008 Jan; 39 (1): 105-10. |
| [18] | Ding J, Zhou D, Geng T, Pan L, Ya J, Wang Z, Hu Y, Ding Y, Zhang X, Meng R. To Predict Visual Deterioration According to the Degree of Intracranial Hypertension in Patients with Cerebral Venous Sinus Thrombosis. Eur Neurol. 2018; 80 (1-2): 28-33. |
APA Style
Maliha Hakim, Mohammad Nur Uddin, Fatema Ahmed, Mashfiqul Hasan. (2021). Cerebral Venous Sinus Thrombosis in a 40-year-old Lady with JAK2-positive Polycythemia Vera: A Case Report. Clinical Neurology and Neuroscience, 5(3), 60-62. https://doi.org/10.11648/j.cnn.20210503.15
ACS Style
Maliha Hakim; Mohammad Nur Uddin; Fatema Ahmed; Mashfiqul Hasan. Cerebral Venous Sinus Thrombosis in a 40-year-old Lady with JAK2-positive Polycythemia Vera: A Case Report. Clin. Neurol. Neurosci. 2021, 5(3), 60-62. doi: 10.11648/j.cnn.20210503.15
AMA Style
Maliha Hakim, Mohammad Nur Uddin, Fatema Ahmed, Mashfiqul Hasan. Cerebral Venous Sinus Thrombosis in a 40-year-old Lady with JAK2-positive Polycythemia Vera: A Case Report. Clin Neurol Neurosci. 2021;5(3):60-62. doi: 10.11648/j.cnn.20210503.15
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Download@article{10.11648/j.cnn.20210503.15,
author = {Maliha Hakim and Mohammad Nur Uddin and Fatema Ahmed and Mashfiqul Hasan},
title = {Cerebral Venous Sinus Thrombosis in a 40-year-old Lady with JAK2-positive Polycythemia Vera: A Case Report},
journal = {Clinical Neurology and Neuroscience},
volume = {5},
number = {3},
pages = {60-62},
doi = {10.11648/j.cnn.20210503.15},
url = {https://doi.org/10.11648/j.cnn.20210503.15},
eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.cnn.20210503.15},
abstract = {Cerebral venous sinus thrombosis (CVST) has a range of underlying cause. Here a case of CVST with an uncommon etiology is presented and discussed. A 40-year-old female presented with headache for 2 years and progressive visual loss for 4 months. She had conjunctival congestion and bilateral papilloedema with pale disc on right side. Computed tomography (CT) scans and contrast magnetic resonance imaging (MRI) of brain were normal but venography (MRV) revealed widespread thrombosis of dural sinuses including superior sagittal sinus. Cerebrospianal fluid (CSF) pressure was high (360 mm of H2O) while other CSF parameters were normal. She was polycythemic (hemoglobin 18.1 g/dl, hematocrit 60.2%), Bone marrow study revealed pan-myeloid hyperplasia whereas trephine biopsy revealed hypercellular marrow with trilineage expansion suggestive of myeloproliferative disorder possibly polycythemia vera. JAK-2 mutation was also detected. CVST has different patterns of presentations including isolated intracranial hypertension leading to visual loss. It is essential to identify and treat underlying condition like polycythemia vera.},
year = {2021}
}
TY - JOUR T1 - Cerebral Venous Sinus Thrombosis in a 40-year-old Lady with JAK2-positive Polycythemia Vera: A Case Report AU - Maliha Hakim AU - Mohammad Nur Uddin AU - Fatema Ahmed AU - Mashfiqul Hasan Y1 - 2021/08/31 PY - 2021 N1 - https://doi.org/10.11648/j.cnn.20210503.15 DO - 10.11648/j.cnn.20210503.15 T2 - Clinical Neurology and Neuroscience JF - Clinical Neurology and Neuroscience JO - Clinical Neurology and Neuroscience SP - 60 EP - 62 PB - Science Publishing Group SN - 2578-8930 UR - https://doi.org/10.11648/j.cnn.20210503.15 AB - Cerebral venous sinus thrombosis (CVST) has a range of underlying cause. Here a case of CVST with an uncommon etiology is presented and discussed. A 40-year-old female presented with headache for 2 years and progressive visual loss for 4 months. She had conjunctival congestion and bilateral papilloedema with pale disc on right side. Computed tomography (CT) scans and contrast magnetic resonance imaging (MRI) of brain were normal but venography (MRV) revealed widespread thrombosis of dural sinuses including superior sagittal sinus. Cerebrospianal fluid (CSF) pressure was high (360 mm of H2O) while other CSF parameters were normal. She was polycythemic (hemoglobin 18.1 g/dl, hematocrit 60.2%), Bone marrow study revealed pan-myeloid hyperplasia whereas trephine biopsy revealed hypercellular marrow with trilineage expansion suggestive of myeloproliferative disorder possibly polycythemia vera. JAK-2 mutation was also detected. CVST has different patterns of presentations including isolated intracranial hypertension leading to visual loss. It is essential to identify and treat underlying condition like polycythemia vera. VL - 5 IS - 3 ER -
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