臂丛神经淀粉样变性一例

A Case of Localized Amyloidosis of the Brachial Plexus

局灶性周围神经淀粉样变性是一种罕见的淀粉样变性,好发于中老年人,也可发生于青年人,以局灶性神经症状为主,组织病理检测为诊断金标准,可完善质谱分析或免疫组化明确淀粉类型。本文回顾性分析1例局灶性臂丛神经淀粉样变性患者的临床特点。该患者女性,34岁,发现右颈部肿块2年,右上肢无力2个月。臂丛MRI见右侧颈5与颈6神经根部及上干瘤样改变。肌电图见右侧臂丛神经上干损伤。臂丛神经活检发现刚果红染色阳性淀粉样物质沉积。组织质谱分析明确淀粉类型为AHL型(G-λ)型。明确诊断后,该患者行神经移植治疗。同时对局灶性脊神经淀粉样变性的国内外相关文献资料进行复习,发现该类疾病平均起病年龄为62.4岁,桡神经是最容易受累的部位,其次为腰骶丛,50%的淀粉类型为AL型,目前尚无统一的治疗方案。现对局灶性周围神经淀粉样变性的临床特点进行总结,以提高对该疾病的认识。

Localized peripheral neuropathy amyloidosis is a rare disease that mainly occurred in elder people who present with focal neurological symptoms. AL is the main type of amyloid protein. Biopsy is the golden standard for diagnosis. Mass spectrometry and immunohistochemical analysis help to confirm the type of amyloid protein. This paper retrospectively analyzes the clinical and imaging data, auxiliary examinations, histological, and immunohistochemical markers. The patient, a 34-year-old woman, presented with a right neck mass and weakness of the right arm. Brachial plexus magnetic resonance imaging(MRI) showed a tumor-like lesion in the nerve root at C5 and C6 and in upper trunk. Electrophysiological studies revealed damage in the upper trunk of the brachial plexus. Positive staining with Congo red was found in brachial plexus biopsy. Mass spectrometry showed that the type of amyloid protein was AHL(G-λ).The patient underwent nerve graft for treatment. Meanwhile, literature review revealed that the average onset age of localizedspinal nerve amyloidosis was 62.4 years old.The radial nerve was the most susceptible, followed by the lumbosacral plexus. Fifty percent of the type of amyloid protein is AL.Until now, no consolidated treatment is available. Here, we summarize the clinical characteristics of localized peripheral neuropathy amyloidosis in order to raise the awareness of the disease.