干燥综合征患者伴神经系统损害7例临床及病理变化

Clinical and pathological changes of neuromuscular complications in 7 Sjogren's syndrome cases

目的 探讨干燥综合征患者合并肌肉及周围神经损害患者的临床及其肌肉和周围神经病理改变。方法 对 7例患者的临床表现、实验室检查进行系统描述 ,对肌肉及神经组织进行组织学及组织化学染色 ,并行电子显微镜检查。结果 2例神经组织活检可见有髓神经纤维严重脱失 ,有髓神经纤维数目严重减少 ,轻度轴索变性。 5例肌肉活检可见肌纤维萎缩、坏死 ,血管周围有单核样细胞浸润 ,肌内衣有微血管炎的病理改变。结论 干燥综合征患者的肌肉或周围神经并发症在疾病的早期即可以出现 ,肌肉或神经活检可证实有无损害及其严重程度 ,早期诊断及早期治疗对改善干燥综合征合并神经系统损害患者的预后是很有必要的。

Objective To study the clinical features and pathological changes of muscles and peripheral nerves in Sjogren's syndrome cases accompanied with neuromuscular complications. Methods The muscle and peripheral nerve biopsies of 7 cases were performed and the specimens were tested by using histochemical and electron microscopic methods. Results The results revealed that myelinated fiber number in peripheral nerves was severely decreased in 2 cases, and the manifestations of atrophy, necrosis, vasculitis and mononuclear cell inflitration could be found in the muscle tissues in 5 cases. Conclusions The neurologic complications of Sjogren's syndrome appear commonly and early. The muscle and peripheral nerve biopsies indicate that complications and its degree,in patients with Sjogren's syndrome should be early diagnosed and treated.