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以脑静脉血栓形成表现的原发性干燥综合征:1例罕见病例

Primary Sjögren's Syndrome Presenting as Cerebral Venous Thrombosis: A Rare Case.

作者信息

Chandra Mohan Sonu, G A Monisha, M Ravi Kiran

机构信息

Internal Medicine, Mysore Medical College and Research Institute, Mysuru, IND.

出版信息

Cureus. 2022 Sep 4;14(9):e28772. doi: 10.7759/cureus.28772. eCollection 2022 Sep.

Abstract

Sjogren's syndrome is a late-onset, slowly progressing autoimmune disease characterized by the destruction of the exocrine glands by lymphocytic infiltration, resulting in dry mouth (xerostomia) and dry eyes (keratoconjunctivitis sicca). Sjögren's syndrome may be associated with various autoimmune diseases, including systemic lupus erythematosus, rheumatoid arthritis, and systemic sclerosis. We report a case of a 34-year-old female who delivered a live baby 20 days ago. She presented in a postictal state after two episodes of tonic-clonic movements of limbs with altered sensorium with a history of headache for seven days. Further evaluation revealed that the subject had a history of multiple abortions and grittiness in her eyes. MRI showed signs of infarction in the left parietal lobe and magnetic resonance venography (MRV) suggested cavernous venous thrombosis. After an unwavering effort to rule out alternate causes, the rare correlation between primary Sjogren's syndrome and cerebral venous thrombosis was considered. Additional investigations were performed, which showed the patient to be positive for Anti SS-A (Ro52), Anti SS-B (La), and anti-centromere antibodies. The patient gradually improved with anti-edema measures and steroids and was discharged by day nine. We present this case to emphasize the neurological manifestation of Sjogren's syndrome, which may present as cerebral venous thrombosis.

摘要

干燥综合征是一种起病较晚、进展缓慢的自身免疫性疾病,其特征是淋巴细胞浸润导致外分泌腺破坏,从而引起口干(口干燥症)和眼干(干燥性角结膜炎)。干燥综合征可能与多种自身免疫性疾病相关,包括系统性红斑狼疮、类风湿关节炎和系统性硬化症。我们报告一例34岁女性病例,该女性20天前分娩一活婴。她在出现两次肢体强直阵挛运动且意识改变后呈发作后状态,有7天头痛病史。进一步评估发现该患者有多次流产史且眼睛有沙砾感。磁共振成像(MRI)显示左顶叶有梗死迹象,磁共振静脉血管造影(MRV)提示海绵窦静脉血栓形成。在经过不懈努力排除其他病因后,考虑到原发性干燥综合征与脑静脉血栓形成之间的罕见关联。进行了进一步检查,结果显示患者抗SS - A(Ro52)、抗SS - B(La)和抗着丝点抗体呈阳性。患者通过抗水肿措施和类固醇治疗后逐渐好转,并在第9天出院。我们展示此病例以强调干燥综合征的神经学表现,其可能表现为脑静脉血栓形成。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5fd5/9531848/f721147c6c38/cureus-0014-00000028772-i01.jpg

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