家族性地中海热和中风的共存

M. Batum, A. Kısabay, M. Akgul, D. Selçuki
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引用次数: 1

摘要

家族性地中海热(FMF)是一种遗传性疾病,以发热、腹膜炎、关节炎、丹毒形式的皮肤病变为特征,并伴有反复发热和常染色体隐性自身免疫特征。FMF是一种炎症性疾病,当促凝因子在发作期间和发作间期增加时,抗凝血和纤溶活性降低。因此,容易形成血栓。46岁女性患者,因急性FMF发作在风湿病科随访时,因口舌右移、言语障碍、左侧无力、麻木入住卒中病房。患者脑部及弥散性磁共振(MR)成像显示右侧大脑中动脉灌注区弥散受限,随访发现右侧基底节水平梗死内血肿。由于两种临床形式并存,认为该病例适用于缺血性脑卒中症状,并在随访中出现梗死内血肿的情况是合适的。文献中未见两种不同临床症状共存的类似病例。
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The Coexistence of Familial Mediterranean Fever and Stroke
Familial Mediterranean Fever (FMF) is a hereditary disease that is characterized by fever, peritonitis, arthritis, skin lesions in the form of erysipelas and has recurrent episodes of fever and features of autosomal recessive autoimmunity. In FMF, which is an inflammatory disease, while procoagulant factors increase both during episodes and inter-episode periods, the anticoagulant and fibrinolytic activity decrease. As a result, predisposition to thrombosis occurs. A 46-year-old female patient, while she was being followed-up because of acute FMF episode in the Rheumatology department, she was admitted to the stroke unit due to the right shift of the mouth, speech disorder, the left side weakness and numbness. In the brain and diffusion Magnetic Resonance (MR) imaging of the patient, it was seen that there was the diffusion restriction in the field irrigated by right middle cerebral artery, and the intra-infarct hematoma on right side at the level of basal ganglia in follow-up. It was thought to be appropriate to present the case, which applied with ischemic stroke symptom and had development of intra-infarct hematoma in follow-up, due to the coexistence of two clinical forms. There were no similar cases that had coexistence of two different clinical symptoms, in the literature.
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