目的:探讨系统性红斑狼疮伴颅内弥漫性钙化的临床特点。
方法:分析1例系统性红斑狼疮伴颅内弥漫性钙化的临床特点。通过Medline和万方数据库检索12例相关文献。
结果:我们的病例和所审查的12例病例均为女性。除了一个案例,SLE的病程超过5年。神经系统的临床表现多样,包括癫痫,偏瘫,认知障碍,和精神异常。在存在神经精神表现的情况下,该病例和6例复查的病例均有SLE活动.对7例患者进行了脑脊液(CSF)检查,包括四名脑脊液蛋白升高的患者,两名IL-6升高的患者,和一名抗核糖体p抗体升高的患者。该病例和12例病例中的10例有双侧基底节钙化。CT显示颅内钙化密度很高,MRI显示T1WI高,T2WI低。
结论:系统性红斑狼疮伴颅内钙化是SLE的一种罕见且严重的表现,这并不完全平行于SLE活动。神经系统的临床表现多样,而双侧基底节钙化在影像学上最为常见。高T1WI信号和低T2WI信号可以作为识别颅内钙化的成像特征之一。
OBJECTIVE: to investigate the clinical characteristics of systemic lupus erythematosus with diffuse intracranial calcification.
METHODS: The clinical characteristics of one case of systemic lupus erythematosus with diffuse intracranial calcification were analyzed, and 12 cases in related literatures were reviewed by searching Medline and Wanfang database.
RESULTS: Our case and 12 cases reviewed were all female. With the exception of one case, the course of SLE was more than 5 years. The clinical manifestations of the nervous system are diverse, including epilepsy, hemiplegia, cognitive impairment, and mental abnormalities. In the presence of neuropsychiatric manifestations, this case and six cases reviewed had SLE activity. Cerebrospinal fluid (CSF) examination was performed in seven patients, including four patients with CSF protein elevation, two patients with IL-6 elevation, and one patient with anti-ribosomal p antibody elevation. This case and 10 of 12 cases reviewed had bilateral basal ganglia calcification. Intracranial calcification was very high density on CT and showed high T1WI and low T2WI signal on MRI.
CONCLUSIONS: Systemic lupus erythematosus with intracranial calcification is a rare and severe manifestation of SLE, which is not completely parallel to SLE activity. The clinical manifestations of the nervous system are diverse, and bilateral basal ganglia calcification is the most common in imaging. High T1WI signal and low T2WI signal may be used as one of the imaging features to identify intracranial calcification.