类淀粉变性多神经病

目的　探讨类淀粉性周围神经病的临床特点及病理改变。方法　报告 3例经腓肠神经和皮肤活检证实的病例 ,结合文献分析其临床特点、病理改变及治疗。结果　本病为慢性进行性周围神经病 ,可伴有心、胃肠、肾等损伤。病理改变示刚果红染色阳性 ,可见类淀粉物沉积于神经内膜、束膜及血管壁外膜 ,有髓及无髓纤维减少 ,节段性脱髓鞘。结论　根据临床特点及周围神经皮肤活检进行诊断 ,肝移植是治疗本病最有前途的方法

Amyloid polyneuropathy

Objective To explore the clinical features and pathologyical changes of amyloid polyneuropathy. Methods Report 3 cases proven by biopsy of the sural nerve and skin,and analyzed with review of literatures that clinical features,pathological changes and treatment of this diseases. Results Amyloid polyneuropathy is chronic progressive peripheral neuropathy,can be accompanied by injured of cardiovascular system,gastrointestinal tract and kidneys;pathological changes showed:Kongorot staining positive,amyloid deposited in endoneurium,perineurium and vascular adventitia,a marked reduction in the number of myelinated fibers and unmyelinated fibers,segmental demylinated. Conclusion Diagnose this disease according to clinical features and biopsy of peripheral nerves and skin,the liver transplantation is an future treatment for amyloid polyneuropathy.