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一名横窦血栓形成患者抗磷脂综合征与蛋白S缺乏症并存:一种罕见的关联。

Coexistence Between Antiphospholipid Syndrome and Protein S Deficiency in a Patient With Transverse Sinus Thrombosis: A Rare Association.

作者信息

Mahmoud Ziryab Imad Taha, Abdalla Yassin Abdelrahim, Omer Hoyam Bakri Gafar Elhaj, Ali Obada Mohamed Ahmed, Suliman Muhammad Salah Ali, Awad Asma, Hamza Salih Boushra, Elmobark Sara Omar Elamin, Gafar Mohammedelmuntaga, Osman Abuobieda Omer

机构信息

University of Bahri Khartoum Sudan.

Ziryab Research Group Khartoum Sudan.

出版信息

Clin Case Rep. 2024 Dec 5;12(12):e9663. doi: 10.1002/ccr3.9663. eCollection 2024 Dec.

Abstract

The primary antiphospholipid syndrome and protein S deficiency are known hypercoagulable states predisposing to strokes. We present a 34-year-old woman presented to rheumatology clinic complaining of right side weakness and aphasia for 2 months before the visit. There was joint pain in the right elbow and shoulder joints, hyperpigmentation on her face and dry painful red eyes mainly the right eye in addition to dry mouth. She had a history of recurrent abortions. Neurological examination showed hypertonia on right lower and upper limb and normal on left one, while the power was grade 3 on right side and normal in left one. Upper motor neuron signs and facial palsy was noted. Hyperpigmentation in the face was observed. MRI brain showed that left temporoparietal hemorrhagic infraction involving the basal ganglia and MRV brain showed left transverse sinus thrombosis and attenuation of sigmoid and internal jugular vein. B2-glycoprotein Ig AGM, lupus anticoagulant, anticardiolipin (IgG, IgM, and IgA), protein S were positive. ANA profile was borderline for PCNA. We report unusual venous sinus thrombosis with primary antiphospholipid syndrome and acquired protein S deficiency.

摘要

原发性抗磷脂综合征和蛋白S缺乏是已知的易引发中风的高凝状态。我们报告了一名34岁女性,在就诊前2个月因右侧肢体无力和失语前往风湿病诊所就诊。除口干外,她的右肘和肩关节疼痛、面部色素沉着、双眼干涩疼痛,主要是右眼。她有反复流产史。神经系统检查显示右下肢和上肢肌张力增高,左下肢和上肢正常,右侧肌力为3级,左侧正常。存在上运动神经元体征和面瘫。观察到面部色素沉着。脑部MRI显示左颞顶叶出血性梗死累及基底节,脑部MRV显示左横窦血栓形成,乙状窦和颈内静脉变细。B2-糖蛋白Ig AGM、狼疮抗凝物、抗心磷脂(IgG、IgM和IgA)、蛋白S均为阳性。ANA谱中PCNA处于临界值。我们报告了原发性抗磷脂综合征合并获得性蛋白S缺乏导致的不寻常静脉窦血栓形成。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa4c/11620977/71d6d94bd994/CCR3-12-e9663-g002.jpg

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