神经淋巴瘤病 (NL) 的特征是周围神经系统的淋巴瘤浸润，表现为淋巴瘤的初始表现（原发性 NL [PNL]）或已知淋巴瘤的复发（继发性 NL [SNL]）。本报告详细介绍并比较了这2组患者的神经临床病理特征。这项回顾性研究对1992年1月1日至2020年6月31日期间经病理证实的神经NL患者进行。患者的临床特征、神经系统检查、影像学研究、收集、分析和比较 PNL 和 SNL 的 EMG 和神经活检数据。总共确定了 58 名患者（34 名 PNL 和 24 名 SNL）。 PNL 中从神经系统症状出现到诊断的时间较长，为 18.5 个月，而 SNL 为 5.5 个月 (p = 0.01)。两组患者的神经系统症状相似，主要包括感觉丧失（98%）、剧烈疼痛（76%）和不对称无力（76%）。观察到 EMG 证实的多种不同神经病变模式，但与 PNL 患者相比（n = 1，p = 0.01），SNL 患者的单神经病变数量增加（n = 8）。与氟脱氧葡萄糖 (FDG)-PET CT 成像研究 (60%) 相比，MRI 研究更频繁地检测到 NL (86%) (p = 0.007)。神经活检显示 B 细胞淋巴瘤（PNL n = 32，SNL n = 22），其次是 T 细胞淋巴瘤（PNL n = 2，SNL n = 2），两组脱髓鞘增加，轴突变性增加（p = 0.01）和多灶有髓纤维损失（p = 0.04）在 SNL 与 PNL 中显着。识别 SNL 会导致患者治疗方法发生改变，但与 PNL 相比预后更差 (p = 0.025)。虽然 PNL 和 SNL 都是主要疼痛且不对称的神经病，在 EMG 和神经活检中均具有轴索和脱髓鞘特征，但 SNL 与暴发性 PNL 的表现有些不同。与 FDG-PET/CT 相比，MRI 可以更好地检测不对称的单神经病。 SNL 的局灶性模式可能是残留癌细胞逃避初始化疗的结果，这些化疗不会穿过血神经屏障，这些细胞随后可能复发并导致暴发性疾病。尽管仍然导致预后较差，但识别 SNL 很重要，因为这改变了每个 SNL 病例的治疗和管理。

Neurolymphomatosis (NL) is characterized by lymphomatous infiltration of the peripheral nervous system presenting as the initial manifestation of a lymphoma (primary NL [PNL]) or in relapse of a known lymphoma (secondary NL [SNL]). This report details and compares the neurologic clinicopathologic characteristics of these 2 groups.This retrospective study was performed on patients diagnosed with pathologically confirmed NL in nerve between January 1, 1992, and June 31, 2020. Patient clinical characteristics, neurologic examination, imaging studies, EMG, and nerve biopsy data were collected, analyzed, and compared between PNL and SNL.A total of 58 patients were identified (34 PNL and 24 SNL). Time from neurologic symptom onset to diagnosis was longer in PNL at 18.5 months compared with 5.5 months in SNL (p = 0.01). Neurologic symptoms were similar in both patient groups and included primarily sensory loss (98%), severe pain (76%), and asymmetric weakness (76%). A wide spectrum of EMG-confirmed different neuropathy patterns were observed, but patients with SNL had increased numbers of mononeuropathies (n = 8) compared with PNL (n = 1, p = 0.01). MRI studies detected NL more frequently (86%) compared with fluorodeoxyglucose (FDG)-PET CT imaging studies (60%) (p = 0.007). Nerve biopsies revealed B-cell lymphoma (PNL n = 32, SNL n = 22), followed by T-cell lymphoma (PNL n = 2, SNL n = 2), with increased demyelination in both groups and increased axonal degeneration (p = 0.01) and multifocal myelinated fiber loss (p = 0.04) significant in SNL vs PNL. Identifying SNL resulted in patient treatment modifications but a worse prognosis compared with PNL (p = 0.025).While PNL and SNL are both primarily painful and asymmetric neuropathies with axonal and demyelinating features on EMG and nerve biopsy, SNL presents somewhat differently than PNL with fulminant, asymmetric often mononeuropathies better detected on MRI than FDG-PET/CT. The focal pattern of SNL is likely a result of residual cancer cells that evaded initial chemotherapy, which does not cross the blood-nerve barrier, and these cells can later recur and result in fulminant disease. Although still resulting in a poorer prognosis, identifying SNL is important because this changed treatment and management in every SNL case.