摘要目的:分析神经痛性肌萎缩（NA）患者的临床特征和神经电生理特点，探讨其神经功能状况。方法:收集2016年9月至2024年1月在清华大学附属北京清华长庚医院确诊的90例NA患者的临床资料和神经电生理数据，进行系统回顾和分析，对其临床表型及神经电生理特点进行总结。结果:在90例患者中，男性占60%（54例），女性占40%（36例）。病程为12（3，36）个月（1周至5年）。患者的发病年龄为58（30，70）（21~87）岁。94.4%（85/90）的患者表现为单侧病变，左侧∶右侧为1∶1.3，仅5.6%（5/90）为双侧患病。患者多呈单相病程，仅有2.2%（2/90）复发。70.0%（63/90）的患者表现为上肢疼痛，病程1 d至1个月内可出现肌无力和肌肉萎缩；而30.0%（27/90）的患者无明显临床疼痛症状。病变主要累及臂丛的上干［64.4%（58/90）］。14.4%（13/90）的患者表现为上肢远端神经损伤，其中6.7%（6/90）为孤立性前骨间神经受累，6.7%（6/90）为孤立性后骨间神经受累，1例同时存在前、后骨间神经损伤。另有1例患者表现为双侧膈神经受累，1例为孤立性胫后神经损伤。神经电生理检查发现运动神经纤维轴索损伤是NA电生理表现的主要特征。患者运动神经传导表现为复合肌肉动作电位波幅下降［68.8%（55/80）］和潜伏期延长［31.3%（25/80）］，感觉神经传导无异常［60.0%（48/80）］或表现为潜伏期延长［15.0%（12/80）］、波幅降低［12.5%（10/80）］、传导速度减慢［8.8%（7/80）］及波形未引出［3.8%（3/80）］。NA患者的神经传导检测在运动神经中以肩胛上神经异常最常见［70.6%（36/51）］，其次是腋神经［58.3%（35/60）］、肌皮神经［50.7%（35/69）］、胸长神经（6/17）、前骨间神经［7.5%（6/80）］和后骨间神经［7.5%（6/80）］。在感觉神经中以前臂外侧皮神经异常为主［30.0%（12/40）］。针极肌电图表现为神经源性损害，以冈下肌［69.2%（18/26）］、肱二头肌［68.1%（49/72）］、三角肌［65.3%（47/72）］病变多见。NA患者的磁共振神经成像（MRN）阳性率为62.1%（41/66），其中63.4%（26/41）表现为臂丛神经局部肿胀，51.2%（21/41）表现为T 2高信号，4.9%（2/41）表现为肌肉失神经改变。NA神经超声的阳性率为71.1%（59/83），其中91.5%（54/59）表现为神经肿胀，8.5%（5/59）表现出沙漏样收缩。 结论:NA是一种以自发疼痛、肢体无力和（或）肌肉萎缩为主要临床表现的周围神经病变。其临床表型以臂丛上干损伤为主，也可表现为孤立性单神经病变。神经电生理以臂丛上干所支配肌肉神经源性损害最为常见，以运动神经轴索损害为主，亦可表现为纯运动神经受损。MRN及神经超声可帮助定性诊断。

abstractsObjective:To analyze the clinical characteristics and neurophysiological features of patients with neuralgic amyotrophy (NA) and explore their neurological function status.Methods:Clinical data and neurophysiological findings of 90 patients diagnosed with NA at Beijing Tsinghua Changgung Hospital from September 2016 to January 2024 were collected and their clinical phenotypes and neurophysiological characteristics were systematically summarized and analyzed.Results:Among the 90 patients, males accounted for 60.0% (54 cases) and females accounted for 40.0% (36 cases). The duration of the disease was 12 (3, 36) months (ranged from 1 week to 5 years). The onset age of the patients was 58 (30, 70) (21-87) years. Unilateral involvement was noted in 94.4% (85/90) of patients, exhibiting a left-to-right ratio of 1∶1.3, while only 5.6% (5/90) had bilateral involvement. The majority of patients demonstrated a monophasic clinical course with a recurrence rate of just 2.2% (2/90). The primary clinical manifestations included upper limb pain in 70.0% (63/90) of patients, which progressed to muscle weakness and atrophy within 1 day to 1 month, whereas 30.0% (27/90) of patients without significant pain symptoms. Lesions predominantly affected the upper trunk of the brachial plexus, which accounted for 64.4% (58/90) of patients. Distal nerve injuries in the upper limb were observed in 14.4% (13/90) of patients, with 6.7% (6/90) demonstrating isolated anterior interosseous nerve involvement and another 6.7% (6/90) exhibiting isolated posterior interosseous nerve involvement; 1 case had concurrent anterior and posterior interosseous nerve damage. Additionally, 1 case presented with bilateral phrenic nerve involvement, and another patient had isolated posterior tibial nerve injury. Electrophysiological evaluations of patients with NA revealed that axonal damage to motor nerve fibers was a hallmark feature of the condition. Among patients undergoing motor nerve conduction studies, 68.8% (55/80) exhibited decreased compound muscle action potential amplitude, and 31.3% (25/80) had prolonged latency. Sensory nerve conduction was normal in 60.0% (48/80) of patients, while abnormalities included prolonged latency in 15.0% (12/80), reduced amplitude in 12.5% (10/80), slowed conduction velocity in 8.8% (7/80), and absent waveforms in 3.8% (3/80) of patients. The rates of abnormal nerve conduction findings in motor nerves were the highest in the suprascapular nerve (70.6%, 36/51), followed by the axillary nerve (58.3%, 35/60), musculocutaneous nerve (50.7%, 35/69), long thoracic nerve (6/17), and both anterior and posterior interosseous nerves (7.5%, 6/80 each). In sensory nerves, abnormalities were predominantly noted in the lateral antebrachial cutaneous nerve (30.0%, 12/40). Needle electromyography demonstrated neurogenic damage, most frequently affecting the infraspinatus muscle (69.2%, 18/26), biceps brachii (68.1%, 49/72), and deltoid muscle (65.3%, 47/72). The positive rate of magnetic resonance neurography (MRN) for NA was 62.1% (41/66), among which 63.4% (26/41) showed localized swelling of the brachial plexus, 51.2% (21/41) exhibited T 2 hyperintensity, and 4.9% (2/41) demonstrated denervated changes in the muscles. The positive rate of ultrasound for NA was 71.1% (59/83), with 91.5% (54/59) showing nerve swelling and 8.5% (5/59) exhibiting hourglass constriction .Conclusions:NA is a peripheral neuropathy characterized by spontaneous pain, limb weakness, and (or) muscle atrophy primarily. Its clinical phenotype predominantly involves damage to the upper trunk of the brachial plexus, which can also manifest as isolated mononeuropathy. Neurophysiological findings most commonly reveal the neurogenic damage to the muscles innervated by the upper trunk of the brachial plexus, mainly characterized by the axonal damage to the motor nerves, and pure motor nerve damage may also be observed. MRN and neuroultrasound can assist in qualitative diagnosis.