Cerebral Sinus Thrombosis in Scleroderma: A Case Report
Abstract
Scleroderma or systemic sclerosis is a multisystem disease due to excessive collagen deposition in different organs and autoimmunity by production of autoantibodies.According to previous reports, brain is rarely affected in scleroderma, howeverrecent studies show central nervous system can be affect not only as a complication of systemic involvement (hypertension, renal failure) but also as a primary manifestation.In scleroderma, thrombus formation in central nervous system and peripheral systems is uncommonmay be due to endothelial cells damage which causes to release antithrombotic factors. We discuss a scleroderma patient with high titter of anticardiolipin antibody who developed to cerebral sinus thrombosis and cerebellum infarction. Then we review literature for both primary brain involvement and thrombotic event in systemic sclerosis.
Terrier B, Charbonneau F, Touzé E, Berezne A, Pagnoux C, Silvera S, Meder JF, Guillevin L, Oppenheim C, Mouthon L.Cerebral vasculopathy is associated with severe vascular manifestations in systemic sclerosis. J Rheumatol 2009;36(7):1486-94.
Ahathya RS, Deepalakshmi D, Emmadi P.Systemic sclerosis. Indian J Dent Res 2007;18(1):27-30.
Giuliodori G, Fraticelli P, Bartolini M, Cagnetti C, Baruffaldi R, Rocchi MB, Provinciali L, Gabrielli A, Silvestrini M. Cognitive and cerebral hemodynamic impairment in scleroderma patients. Eur J Neurol 2009;16(12):1285-90
Estey E, Lieberman A, Pinto R, Meltzer M, Ransohoff J. Cerebral arteritis in scleroderma. Stroke 1979;10(5):595-7.
Mohamed RH, Nassef AA. Brain magnetic resonance imaging findings in patients with systemic sclerosis. Int J Rheum Dis 2010;13(1):61-7.
Heron E, Hernigou A, Chatellier G, Fornes P, Emmerich J, Fiessinger JN. Intracerebralcalcification in systemic sclerosis. Stroke 1999;30(10):2183-5.
Das CP, Prabhakar S, Lal V, Kharbanda PS.Scleroderma, stroke, optic neuropathy: a rare association. Neurol India 2002;50(4):504-7.
Chung L, Utz PJ.Antibodies in scleroderma: directpathogenicity and phenotypic associations. Curr Rheumatol Rep 2004;6(2):156-63.
Gupta R, Thabah MM, Gupta S, Shankar S, Kumar A.Clinical significance of antiphospholipid antibodies in Indian scleroderma patients. Rheumatol Int 2009; 30(2):277-9.
Pathak R, Gabor AJ.Scleroderma and central nervous system vasculitis. Stroke 1991;22(3):410-3.
Kaku Y, Kouda K, Yoshimura S, Sakai N. Cerebral aneurysms in scleroderma. Cerebrovasc Dis 2004; 17(4):339-41.
Files | ||
Issue | Vol 50, No 4 (2012) | |
Section | Original Article(s) | |
Keywords | ||
Scleroderma systemic Cerebral sinus thrombosis Cerebellum infarction |
Rights and permissions | |
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. |