Background: Neuralgic amyotrophy (NA), also known as Parsonage–Turner syndrome, brachial neuritis, and idiopathic brachial plexopathy, is a rare and potentially debilitating peripheral nerve disorder characterized by acute-onset shoulder pain followed by progressive motor deficits. It is often under-recognized, with an estimated incidence of 1 to 3 per 100,000 annually, though some studies suggest the actual prevalence may be significantly higher. The condition typically progresses through three phases, an acute painful phase, a phase of weakness, and a recovery phase, with sensory disturbances common in addition to motor weakness. The exact pathogenesis of NA remains unclear, though it is thought to involve a combination of genetic, environmental, and immunological factors. While neurologic complications following hematopoietic stem cell transplantation (HSCT), such as neuropathies and myopathies, have been documented, NA remains exceedingly rare in this context, with only a few reported cases. The pathophysiology in HSCT patients is hypothesized to involve immune dysregulation, graft-versus-host disease (GvHD), infection, and the effects of immunosuppressive therapy. Diagnosis is primarily clinical, supported by electrodiagnostic studies and MRI, though no laboratory markers exist. The management of NA is largely supportive and multimodal, focusing on pain control and rehabilitation. Objectives: The objective of this study was to describe the characteristics, clinical course, and outcomes of patients admitted for HSCT who were subsequently diagnosed with NA. Study Design: This retrospective case series from a single institution examined nine (N = 9) patients who developed acute shoulder pain following HSCT. We collected data on demographics, transplant details, clinical features, MRI findings, and electrodiagnostic studies, summarized using descriptive statistics. The diagnosis of neurologic amyotrophy was based on clinical presentation and corroborated by imaging and electrodiagnostic results. Long-term follow-up was assessed to evaluate symptom recovery. Results: Between August 2020 and July 2022, nine patients (44% male, median age 60) were diagnosed with NA following autologous (n = 4) or allogeneic (n = 5) HSCT. The onset of severe shoulder pain occurred at a median of 9 days post-transplant (range 1–21 days), with the majority of patients experiencing unilateral pain, predominantly affecting the right shoulder (55%). Neurologic weakness developed on average 5.1 days after pain onset, and sensory deficits were observed in all but one patient. MRI findings revealed muscle edema, atrophy, and enhancement in six patients, while electromyography confirmed NA in five. Due to the small sample size, statistical analyses, includingp-values, confidence intervals, and trend comparisons, were not performed, and thus no conclusions can be drawn regarding associations between variables such as early onset and worse outcomes. Shoulder pain resolved after a median of 23 days (range 8–40 days). Long-term follow-up (>1 year) showed that three patients achieved full or near-full recovery, four partially recovered, and two showed minimal improvement.Conclusions: NA should be highly suspected in patients with acute shoulder pain and neurologic symptoms post-HSCT. To improve diagnostic accuracy and clinical outcomes, we recommend enhanced clinician awareness, the implementation of targeted diagnostic protocols (such as MRI and electrodiagnostic studies), and the establishment of standardized long-term follow-up protocols.
背景：神经痛性肌萎缩（NA），又称Parsonage-Turner综合征、臂丛神经炎或特发性臂丛神经病，是一种罕见且可能致残的外周神经疾病，其特征为急性发作的肩部疼痛，随后出现进行性运动功能障碍。该疾病常被忽视，年发病率估计为每10万人1至3例，但部分研究表明实际患病率可能显著更高。NA通常经历急性疼痛期、无力期和恢复期三个阶段，除运动无力外常伴有感觉障碍。其确切发病机制尚不明确，目前认为涉及遗传、环境和免疫因素的综合作用。尽管造血干细胞移植（HSCT）后的神经系统并发症（如神经病变和肌病）已有记载，但NA在此背景下仍极为罕见，仅有少数病例报道。HSCT患者发生NA的病理生理机制被推测与免疫失调、移植物抗宿主病（GvHD）、感染及免疫抑制治疗的影响相关。诊断主要依靠临床表现，辅以电诊断学和MRI检查，目前尚无特异性实验室标志物。NA的治疗以支持性和多模式综合管理为主，侧重于疼痛控制和康复训练。 目的：本研究旨在描述因HSCT入院后确诊NA患者的临床特征、病程及预后。 研究设计：本单中心回顾性病例系列研究纳入了9例（N=9）HSCT后出现急性肩痛的患者。通过描述性统计方法，收集并分析了人口统计学特征、移植细节、临床表现、MRI结果及电诊断学检查数据。神经痛性肌萎缩的诊断基于临床表现，并经影像学和电诊断学结果证实。通过长期随访评估症状恢复情况。 结果：2020年8月至2022年7月期间，9例患者（男性占44%，中位年龄60岁）在自体（n=4）或异体（n=5）HSCT后被诊断为NA。严重肩痛的中位发生时间为移植后9天（范围1-21天），多数患者表现为单侧疼痛，以右肩受累为主（55%）。神经性无力平均在疼痛发作后5.1天出现，除1例外所有患者均观察到感觉障碍。6例患者MRI显示肌肉水肿、萎缩及强化征象，5例患者肌电图确诊NA。因样本量较小，未进行包括p值、置信区间和趋势比较在内的统计分析，故无法得出关于早期发病与不良预后等变量间关联的结论。肩部疼痛中位缓解时间为23天（范围8-40天）。长期随访（>1年）显示，3例患者完全或接近完全恢复，4例部分恢复，2例改善不明显。 结论：对于HSCT后出现急性肩痛伴神经症状的患者，应高度怀疑NA。为提高诊断准确性和临床预后，建议加强临床医生对该病的认知，实施针对性诊断方案（如MRI和电诊断学检查），并建立标准化的长期随访机制。
肿瘤(癌症)患者之家