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Cerebral venous thrombosis in an adult with relapsing minimal change disease

      Abstract

      Minimal change disease (MCD) is a well-known cause of fulminant acute nephrotic syndrome (NS) and has been associated with thrombotic complications. We report the case of a 51-year-old woman with previous biopsy-proven MCD in remission who presented with worsening headache and acute confusion shortly after a relapse of the NS and was diagnosed with cerebral venous thrombosis (CVT) complicated by intracranial hemorrhage and midline shift. One month prior, she had been initiated on an oral contraceptive agent during remission of the NS. After initiation of systemic anticoagulation, her condition rapidly deteriorated, and she passed away before being able to undergo catheter-based venous thrombectomy. We conducted a systematic literature review and identified 33 case reports of adults with NS-associated CVT. The most common symptoms were headache (83%), nausea or vomiting (47%), and altered mental status (30%). 64% of patients presented at time of initial diagnosis of the NS and 32% during a relapse. Mean urinary protein excretion was 9.32 g/day and mean serum albumin was 1.8 g/dL. 91% of patients received systemic anticoagulation, and 19% died. The outcome in the remaining cases was favorable with only one report (5%) of residual neurological deficit. Of the available kidney biopsy results, MCD was the most common diagnosis (70%), raising the hypothesis that the fulminant acute onset of the NS might be a predisposing factor for this serious thrombotic complication. Clinicians should have a high index of suspicion for CVT in patients with the NS who present with new-onset neurological symptoms, including headache and nausea.

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      References

        • Vivarelli M.
        • Massella L.
        • Ruggiero B.
        • et al.
        Minimal change disease.
        Clin J Am Soc Nephrol. 2017; 12: 332-345https://doi.org/10.2215/CJN.05000516
        • Robson A.M.
        • Giangiacomo J.
        • Kienstra R.A.
        • et al.
        Normal glomerular permeability and its modification by minimal change nephrotic syndrome.
        J Clin Invest. 1974; 54: 1190-1199https://doi.org/10.1172/JCI107862
        • Mahmoodi B.K.
        • ten Kate M.K.
        • Waanders F.
        • et al.
        High absolute risks and predictors of venous and arterial thromboembolic events in patients with nephrotic syndrome: results from a large retrospective cohort study.
        Circulation. 2008; 117: 224-230https://doi.org/10.1161/CIRCULATIONAHA.107.716951
        • Singhal R.
        • Brimble K.S.
        Thromboembolic complications in the nephrotic syndrome: pathophysiology and clinical management.
        Thromb Res. 2006; 118: 397-407https://doi.org/10.1016/j.thromres.2005.03.030
        • Mercadal L.
        • Montcel S.T.
        • Nochy D.
        • et al.
        Factors affecting outcome and prognosis in membranous lupus nephropathy.
        Nephrol Dial Transplant. 2002; 17: 1771-1778https://doi.org/10.1093/ndt/17.10.1771
        • Loscalzo J.
        Venous thrombosis in the nephrotic syndrome.
        The New Engl j med. 2013; 368: 956-958https://doi.org/10.1056/NEJMcibr1209459
        • Barbour S.J.
        • Greenwald A.
        • Djurdjev O.
        • et al.
        Disease-specific risk of venous thromboembolic events is increased in idiopathic glomerulonephritis.
        Kidney Int. 2012; 81: 190-195https://doi.org/10.1038/ki.2011.312
        • Ochiai S.
        • Minakawa A.
        • Fukuda A.
        • et al.
        Dural arteriovenous fistula following cerebral venous sinus thrombosis in a patient with minimal change disease: a case report.
        Clin Nephrol. 2021; 96: 348-352https://doi.org/10.5414/CN110553
        • Nakayama T.
        • Mitsuno R.
        • Torimitsu T.
        • et al.
        Difficulty in managing nephrotic syndrome-associated cerebral venous thrombosis.
        CEN Case Rep. 2021; 10: 132-138https://doi.org/10.1007/s13730-020-00520-7
        • Downie E.
        • Diep J.
        • Sungala N.
        • et al.
        IgM nephropathy complicated by cerebral venous sinus thrombosis: a case study.
        BMC Nephrol. 2020; 21: 390https://doi.org/10.1186/s12882-020-02048-5
        • Chandra T.
        • Tilstra J.S.
        Cerebral venous sinus thrombosis as the initial presentation of systemic lupus erythematosus.
        Lupus. 2020; 29: 213-215https://doi.org/10.1177/0961203319897120
        • Lee J.K.
        • Murray K.
        • Renati S.A.
        Case report of extensive cerebral venous sinus thrombosis as a presenting sign of relapsing nephrotic syndrome.
        Case Rep Neurol Med. 2019; 20196840240https://doi.org/10.1155/2019/6840240
        • Navarro D.
        • Ferreira A.C.
        • Viana H.
        • et al.
        Cavernous sinus thrombosis in a patient with nephrotic syndrome.
        CEN Case Rep. 2017; 6: 136-139https://doi.org/10.1007/s13730-017-0260-7
        • Wang Y.
        • Meng R.
        • Duan J.
        • et al.
        Nephrotic syndrome may be one of the important etiologies of cerebral venous sinus thrombosis.
        J Stroke Cerebrovasc Dis. 2016; 25: 2415-2422https://doi.org/10.1016/j.jstrokecerebrovasdis.2016.06.013
        • Nagaraju S.P.
        • Bairy M.
        • Attur R.P.
        • et al.
        Cerebral venous thrombosis and secondary polycythemia in a case of nephrotic syndrome.
        Saudi J Kidney Dis Transpl. 2016; 27: 391-394https://doi.org/10.4103/1319-2442.178575
        • Li S.J.
        • Tu Y.M.
        • Zhou C.S.
        • et al.
        Risk factors of venous thromboembolism in focal segmental glomerulosclerosis with nephrotic syndrome.
        Clin Exp Nephrol. 2016; 20: 212-217https://doi.org/10.1007/s10157-015-1149-4
        • Hacifazlioglu C.
        • Arslan E.
        • Arslan E.A.
        • et al.
        Cerebral venous sinus thrombosis with internal jugular venous thrombosis in a male patient with nephrotic syndrome.
        Turk Neurosurg. 2015; 25: 980-983https://doi.org/10.5137/1019-5149.JTN.11355-14.1
        • Afghan Z.
        • Hussain A.
        • Asim M.
        Headache in a high school student - a reminder of fundamental principles of clinical medicine and common pitfalls of cognition.
        Qatar Med J. 2015; 2015: 15https://doi.org/10.5339/qmj.2015.15
        • Iwaki H.
        • Neshige S.
        • Hara N.
        • et al.
        [Cerebral venous thrombosis as a complication of nephrotic syndrome–a case report and literature review].
        Rinsho Shinkeigaku. 2014; 54: 495-501https://doi.org/10.5692/clinicalneurol.54.495
        • Zivanovic Z.
        • Barlinn K.
        • Balucani C.
        • et al.
        Headache and aphasia in a young woman with protein S deficiency and nephrotic syndrome.
        Acta Clin Croat. 2011; 50: 619-621
        • Xu H.
        • Chen K.
        • Lin D.
        • et al.
        Cerebral venous sinus thrombosis in adult nephrotic syndrome.
        Clin Nephrol. 2010; 74: 144-149https://doi.org/10.5414/cnp74144
        • Komaba H.
        • Kadoguchi H.
        • Igaki N.
        • et al.
        Early detection and successful treatment of cerebral venous thrombosis associated with minimal change nephrotic syndrome.
        Clin Nephrol. 2007; 68: 179-181https://doi.org/10.5414/cnp68179
        • Nishi H.
        • Abe A.
        • Kita A.
        • et al.
        Cerebral venous thrombosis in adult nephrotic syndrome due to systemic amyloidosis.
        Clin Nephrol. 2006; 65: 61-64https://doi.org/10.5414/cnp65061
        • Chan K.H.
        • Cheung R.T.
        • Mak W.
        • et al.
        Cerebral venous thrombosis presenting as unilateral headache and visual blurring in a man with nephrotic syndrome.
        Hosp Med. 2004; 65: 54-55https://doi.org/10.12968/hosp.2004.65.1.2422
        • Afsari K.
        • Frank J.
        • Vaksman Y.
        • et al.
        Intracranial venous sinus thrombosis complicating AIDS-associated nephropathy.
        AIDS Read. 2003; 13: 143-148
        • Friemel S.P.
        • Mackey D.W.
        • Fenves A.Z.
        • et al.
        Nephrotic syndrome presenting as dural sinus thrombosis.
        Am J Med. 2002; 113: 258-260https://doi.org/10.1016/s0002-9343(02)01167-1
        • Sung S.F.
        • Jeng J.S.
        • Yip P.K.
        • et al.
        Cerebral venous thrombosis in patients with nephrotic syndrome–case reports.
        Angiology. 1999; 50: 427-432https://doi.org/10.1177/000331979905000510
        • Hirata M.
        • Kuroda M.
        • Koni I.
        [Cerebral venous thrombosis in minimal change nephrotic syndrome].
        Nihon Jinzo Gakkai Shi. 1999; 41: 464-468
        • Moller K.
        • Piculell O.R.
        • Sorribes M.M.
        [Fatal hyperthrombotic condition complicating nephrotic syndrome].
        Ugeskr Laeger. 1997; 159: 6683-6686
        • Urch C.
        • Pusey C.D.
        Sagittal sinus thrombosis in adult minimal change nephrotic syndrome.
        Clin Nephrol. 1996; 45: 131-132
        • Laversuch C.J.
        • Brown M.M.
        • Clifton A.
        • et al.
        Cerebral venous thrombosis and acquired protein S deficiency: an uncommon cause of headache in systemic lupus erythematosus.
        Br J Rheumatol. 1995; 34: 572-575https://doi.org/10.1093/rheumatology/34.6.572
        • Burns A.
        • Wilson E.
        • Harber M.
        • et al.
        Cerebral venous sinus thrombosis in minimal change nephrotic syndrome.
        Nephrol Dial Transplant. 1995; 10: 30-34
        • Barthelemy M.
        • Bousser M.G.
        • Jacobs C.
        [Cerebral venous thrombosis, complication of the nephrotic syndrome (author's transl)].
        Nouv Presse Med. 1980; 9 (Thrombose veineuse cerebrale au cours d'un syndrome nephrotique): 367-369
        • de Freitas G.R.
        • Bogousslavsky J.
        Risk factors of cerebral vein and sinus thrombosis.
        Front Neurol Neurosci. 2008; 23: 23-54https://doi.org/10.1159/000111259
      1. ACOG committee opinion no. 556: postmenopausal estrogen therapy: route of administration and risk of venous thromboembolism.
        Obstet Gynecol. 2013; 121: 887-890https://doi.org/10.1097/01.AOG.0000428645.90795.d9
        • Wiles K.
        • Lightstone L.
        Glomerular Disease in Women.
        Kidney Int Rep. Mar. 2018; 3: 258-270https://doi.org/10.1016/j.ekir.2018.01.010
        • Einhaupl K.
        • Stam J.
        • Bousser M.G.
        • et al.
        EFNS guideline on the treatment of cerebral venous and sinus thrombosis in adult patients.
        Eur J Neurol. 2010; 17: 1229-1235https://doi.org/10.1111/j.1468-1331.2010.03011.x
        • Saposnik G.
        • Barinagarrementeria F.
        • Brown Jr., R.D.
        • et al.
        Diagnosis and management of cerebral venous thrombosis: a statement for healthcare professionals from the american heart association/american stroke association.
        Stroke. 2011; 42: 1158-1192https://doi.org/10.1161/STR.0b013e31820a8364
        • Misra U.K.
        • Kalita J.
        • Chandra S.
        • et al.
        Low molecular weight heparin versus unfractionated heparin in cerebral venous sinus thrombosis: a randomized controlled trial.
        Eur J Neurol. 2012; 19: 1030-1036https://doi.org/10.1111/j.1468-1331.2012.03690.x
        • Afshari D.
        • Moradian N.
        • Nasiri F.
        • et al.
        The efficacy and safety of low-molecular-weight heparin and unfractionated heparin in the treatment of cerebral venous sinus thrombosis.
        Neurosciences (Riyadh). 2015; 20: 357-361https://doi.org/10.17712/nsj.2015.4.20150375
        • Durrani J.
        • Malik F.
        • Ali N.
        • et al.
        To be or not to be a case of heparin resistance.
        J Community Hosp Intern Med Perspect. 2018; 8: 145-148https://doi.org/10.1080/20009666.2018.1466599