Department of Neurology, Hospital de Cascais Dr. José de Almeida, Cascais, Portugal.
Department of Hematology, Portuguese Institute of Oncology of Lisbon, Lisbon, Portugal.
Neurol Sci. 2024 Aug;45(8):4003-4006. doi: 10.1007/s10072-024-07445-4. Epub 2024 Mar 12.
Neurolymphomatosis (NL) describes an infiltration of cranial and peripheral nerves by lymphoma cells, most frequently in non-Hodgkin B-cell lymphoma. This clinical entity is rare and poses a challenging diagnosis. We describe a case of a 64-year-old female patient with NL associated with extra-nodal NK/T-cell lymphoma (ENKTL), nasal type, presenting as a painful progressive mononeuropathy multiplex with an oral cavity lesion. ENKTL is usually associated with Epstein-Barr virus (EBV) infection and rarely affects the central and peripheral nervous system. Lumbar puncture, magnetic resonance imaging (MRI), nerve biopsy, and F-fluorodeoxyglucose positron emission tomography (FDG-PET) help to establish the diagnosis. Thereby, NL should be considered in the differential diagnosis of painful progressive multiple neuropathies, even in patients without previous history of cancer.
神经淋巴瘤病（NL）描述的是淋巴瘤细胞浸润颅神经和周围神经，最常发生于非霍奇金 B 细胞淋巴瘤。这种临床实体较为罕见，诊断具有挑战性。我们描述了一例与结外 NK/T 细胞淋巴瘤（ENKTL）相关的 NL 病例，该患者为女性，64 岁，表现为伴有口腔病变的疼痛性进行性多发性单神经病。ENKTL 通常与 EBV 感染相关，很少累及中枢和周围神经系统。腰椎穿刺、磁共振成像（MRI）、神经活检和 F-氟脱氧葡萄糖正电子发射断层扫描（FDG-PET）有助于明确诊断。因此，NL 应在疼痛性进行性多发性神经病的鉴别诊断中考虑，即使在无既往癌症史的患者中也应如此。
