From the Departments of Neurology (M.P.S., J.M.M.-T., M.L.M., C.J.K., P.J.D., P.J.B.D.), Laboratory Medicine and Pathology (R.L.K., W.R.M.), Hematology (T.M.H., P.B.J., I.N.M., A.K.), Radiology (K.A.), Neurosurgery (R.J.S.), and Quantitative Health Sciences (J.M.), Mayo Clinic, Rochester, MN; and Neurological Institute of Thailand (N.S.), Bangkok.
Neurology. 2024 Sep 24;103(6):e209777. doi: 10.1212/WNL.0000000000209777. Epub 2024 Sep 3.
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 ( = 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, = 0.01). MRI studies detected NL more frequently (86%) compared with fluorodeoxyglucose (FDG)-PET CT imaging studies (60%) ( = 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 ( = 0.01) and multifocal myelinated fiber loss ( = 0.04) significant in SNL vs PNL. Identifying SNL resulted in patient treatment modifications but a worse prognosis compared with PNL ( = 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.
神经淋巴病（NL）的特征是淋巴瘤对周围神经系统的浸润，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]）。本报告详细比较了这两组患者的神经临床病理特征。
本回顾性研究纳入了 1992 年 1 月 1 日至 2020 年 6 月 31 日期间经病理证实神经 NL 的患者。收集患者的临床特征、神经系统检查、影像学研究、肌电图和神经活检数据，并对 PNL 和 SNL 之间的数据进行分析和比较。
共确定了 58 例患者（34 例 PNL 和 24 例 SNL）。PNL 从神经系统症状出现到诊断的时间为 18.5 个月，而 SNL 为 5.5 个月（ = 0.01）。两组患者的神经系统症状相似，主要包括感觉丧失（98%）、严重疼痛（76%）和不对称性无力（76%）。观察到肌电图证实的不同神经病变模式广泛存在，但 SNL 患者的单神经病数量（n = 8）多于 PNL（n = 1， = 0.01）。磁共振成像（MRI）检测 NL 的频率高于氟脱氧葡萄糖（FDG）-正电子发射断层扫描（PET）/CT 成像研究（86%比 60%）（ = 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），两组均存在脱髓鞘和轴突变性增加（ = 0.01），且 SNL 比 PNL 更易出现多灶性髓鞘纤维丢失（ = 0.04）。与 PNL 相比，SNL 可导致患者治疗方式改变，但预后较差（ = 0.025）。
尽管 PNL 和 SNL 都是以疼痛为主要表现，且肌电图和神经活检显示为轴突和脱髓鞘特征的不对称性神经病，但 SNL 与 PNL 表现不同，SNL 表现为发作迅速、不对称，常为单神经病，MRI 比 FDG-PET/CT 更易检测到。SNL 的局灶性模式可能是残留癌细胞的结果，这些癌细胞逃避了初始化疗，而化疗不能穿过血-神经屏障，这些细胞以后可能会复发并导致疾病发作。虽然 SNL 仍导致预后较差，但确定 SNL 很重要，因为这会改变每个 SNL 病例的治疗和管理方式。
