|Year : 2017 | Volume
| Issue : 3 | Page : 347-349
Deep cerebral venous thrombosis as a presenting feature of systemic lupus erythematosus
Chandra Mohan Sharma, Banshi Lal Kumawat, Kaushik Ramanlal Rana, Ankur Garg
Department of Neurology, Sawai Man Singh Medical College, Jaipur, Rajasthan, India
|Date of Web Publication||5-Sep-2017|
Kaushik Ramanlal Rana
Sawai Man Singh Medical College, Jaipur, Rajasthan
Source of Support: None, Conflict of Interest: None
Systemic lupus erythematosus (SLE) is an autoimmune disease with various systemic manifestations. Neurological manifestations include cognitive decline, headache, seizure, stroke, and aseptic meningitis. Cerebral venous sinus thrombosis (CVST) is a very rare manifestation of SLE and occasionally can be the presenting feature of SLE. However, thrombosis of a deep venous system as a presenting feature of SLE is scarcely reported. We report a case of a 42-year-old female who presented with a deep cerebral vein thrombosis and was subsequently diagnosed SLE. The clinician should be aware regarding this uncommon presentation of SLE as CVST. A high index of suspicion is required for early diagnosis as early treatment may prevent a fatal outcome.
Keywords: Autoimmune disease, cerebral venous sinus thrombosis, systemic lupus erythematosus
|How to cite this article:|
Sharma CM, Kumawat BL, Rana KR, Garg A. Deep cerebral venous thrombosis as a presenting feature of systemic lupus erythematosus. Indian J Health Sci Biomed Res 2017;10:347-9
|How to cite this URL:|
Sharma CM, Kumawat BL, Rana KR, Garg A. Deep cerebral venous thrombosis as a presenting feature of systemic lupus erythematosus. Indian J Health Sci Biomed Res [serial online] 2017 [cited 2019 Jun 20];10:347-9. Available from: http://www.ijournalhs.org/text.asp?2017/10/3/347/214009
| Introduction|| |
Systemic lupus erythematosus (SLE) is a heterogeneous, multisystem autoimmune disease. Thrombotic manifestations such as deep venous thrombosis in the lower limb and renal vein and mesenteric vein thrombosis have been described in literature, but cerebral venous sinus thrombosis (CVST) has been scarcely reported. Here, we report a case of involvement of thrombosis of deep cerebral venous system as a presenting feature of SLE.
| Case Report|| |
A 42-year-old female presented with altered sensorium without history of febrile illness, seizure, or drug ingestion. She had a history of multiple joint pain. Her menstrual and obstetric history was unremarkable. On examination, she was stuporous and occasionally responding to verbal command, pupils were bilateral symmetrical and reacting to light, and her plantar response was extensor bilaterally. She also had malar rash over the face. Other systemic examinations were unremarkable.
Magnetic resonance imaging (MRI) brain with venogram showed bilateral thalamic hemorrhagic infarction [Figure 1] and thrombosis of the vein of Galen, deep cerebral vein, and straight sinus [Figure 2].
|Figure 1: Magnetic resonance imaging brain T2 axial image showing hemorrhagic infarction of bilateral thalamus|
Click here to view
|Figure 2: Magnetic resonance venogram of the brain showing thrombosis of vein of Galen, deep cerebral vein, and straight sinus|
Click here to view
Hemogram revealed pancytopenia (Hb - 8.0 g/dl, total leukocyte count - 3800/cumm, lymphocytopenia, platelet count - 1.10 lakh/cumm) with raised erythrocyte sedimentation rate-72 mm at a 1st h, and urine analysis revealed proteinuria (1.5 g/24 h) and hematuria. Other routine investigations including blood sugar, electrolytes, liver function test, and renal function test were within normal limit. Cerebrospinal fluid analysis showed mildly raised protein with normal cell count (protein - 60 mg/dl, sugar - 72 mg/dl, and cell count - 3 cell/cumm).
In view of multisystem involvement (history of multiple joint pain, thrombotic state, malar rash, pancytopenia, and nephritic-range proteinuria), we suspected SLE.
Antinuclear antibody was positive with titer 1:160 and rim pattern, and anti-DsDNA was positive with titer 80 IU/ml. Anticardiolipin antibody was negative. As our patient fulfilled six criteria, diagnosis of SLE was made.
The patient was treated with low molecular weight heparin, antibiotics, intravenous fluid therapy, and methylprednisolone 1 g/day for 5 days.
Unfortunately, in spite of all intensive cares, our patient succumbed to death.
| Discussion|| |
CVTS can occur as a complication of SLE (0.36%) or rarely as the presenting symptoms of SLE. Review of literature has suggested that there are few case studies describing CVST and SLE. Singh et al. have published a study of three patients of CVST as a presenting feature of SLE. One of those patients had deep venous system involvement and other two had superficial venous involvement. Vidailhet et al. and Lee et al. also reported handful cases of CVST in SLE., Wang et al. had described clinical characteristic of CVST in 17 patients with SLE. In this study, only one patient was described as presenting feature of SLE. In above-listed studies, sinuses involved were mainly superior sagittal sinus, transverse sinus, and sigmoid sinus. Our case represents CVST as presenting feature of SLE. The unusual feature in our case was involvement of deep venous system and bilateral thalamic infarction.
There are several mechanisms contributing to the formation of CVST in SLE patients include immune complex-induced vasculitis, antiphospholipid antibody, thrombophilia, hyperfibrinogenemia, and infections of the middle ear, facial skin, or intracalvarium.
MRI brain with venogram is the investigation of choice for diagnosis of CVST.
Treatment of CVST in SLE includes corticosteroid, immunosuppressant such as cyclophosphamide, and anticoagulant therapy. Early diagnosis and intensive therapy may prevent associated complication with CVST.
| Conclusion|| |
Deep CVST is a very rare presentation of SLE. High index of suspicion is required. Early diagnosis and intensive treatment may prevent fatal outcome. Treatment strategy includes anticoagulation and immunosuppressant.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1997;40:1725.
Vidailhet M, Piette JC, Wechsler B, Bousser MG, Brunet P. Cerebral venous thrombosis in systemic lupus erythematosus. Stroke 1990;21:1226-31.
Singh RK, Bhoi SK, Kalita J, Misra UK. Cerebral venous sinus thrombosis presenting feature of systemic lupus erythematosus. J Stroke Cerebrovasc Dis 2017;26:518-22.
Lee MK, Kim JH, Kang HR, Rho HJ, Nam EJ, Kim SW, et al
. Systemic lupus erythematosus complicated with cerebral venous sinus thrombosis: A report of two cases. J Korean Med Sci 2001;16:351-4.
Wang L, Chen H, Zhang Y, Liu W, Zheng W, Zhang X, et al
. Clinical characteristics of cerebral venous sinus thrombosis in patients with systemic lupus erythematosus: A single-centre experience in China. J Immunol Res 2015;2015:540738.
Uziel Y, Laxer RM, Blaser S, Andrew M, Schneider R, Silverman ED. Cerebral vein thrombosis in childhood systemic lupus erythematosus. J Pediatr 1995;126(5 Pt 1):722-7.
Stam J. Thrombosis of the cerebral veins and sinuses. N
Engl J Med 2005;352:1791-8.
Xu H, Chen K, Lin D, Dai L, Chen H, Xu Z. Cerebral venous sinus thrombosis in adult nephrotic syndrome. Clin Nephrol 2010;74:144-9.
Deschiens MA, Conard J, Horellou MH, Ameri A, Preter M, Chedru F, et al
. Coagulation studies, factor V Leiden, and anticardiolipin antibodies in 40 cases of cerebral venous thrombosis. Stroke 1996;27:1724-30.
[Figure 1], [Figure 2]