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目的报道1例干燥综合征继发脑干和长节段脊髓病变病例。方法回顾性描述1例干燥综合征继发脑干和脊髓病变患者的临床资料、实验室和影像学检查结果以及唇腺活检病理,并复习相关文献进行分析。结果患者为青壮年女性,临床表现为急性起病的右侧肢体麻木和无力,伴口齿不清,血清SSA、Ro-52及SSB均阳性,MRI示双侧脑干及脊髓(胸7-胸11)长节段异常信号,唇腺活检示腺体间淋巴细胞浸润伴小血管周围炎。经大剂量激素冲击治疗后患者右侧肢体无力好转。结论干燥综合征引起脑干和脊髓的同期病变病例报道较少,临床需引起重视。详细的病史追溯、血清自身抗体筛查和唇腺活检病理对诊断具有重要意义。
Objective To report a case of Sjogren’s syndrome secondary to brain stem and long segment spinal cord lesions. Methods The clinical data, laboratory and imaging findings of the patients with Sjogren’s syndrome secondary to brain stem and spinal cord lesions and the labial biopsy were retrospectively reviewed. Relevant literatures were reviewed. Results The patients were young and middle-aged women. The clinical manifestations were acute onset of numbness and weakness in the right limb with unclean mouth, positive serum SSA, Ro-52 and SSB, MRI showed bilateral brain stem and spinal cord 11) long segment abnormal signal, labial gland biopsy showed glandular lymphocyte infiltration with small perivascular inflammation. After the treatment of high-dose hormones in patients with right limb weakness improved. Conclusion Sjogren ’s syndrome caused fewer cases of simultaneous lesions of brain stem and spinal cord, which should be paid more attention to. Detailed medical history, serum autoantibody screening and labial biopsy pathology is of great significance for the diagnosis.