肿瘤旁神经病变(PNDs)是一种异质的临床病理综合征，发生在恶性肿瘤或其转移灶远离器官或组织的破坏。严重的神经功能障碍与缓慢恶性肿瘤之间的不一致，表明存在一种基础的神经免疫宿主免疫反应，对神经组织造成损伤，同时抑制恶性肿瘤生长。运动系统受累，其他症状和体征与脑、脊髓、周围神经、神经肌肉连接或肌肉的局部或弥漫性受累有关，这是由于潜在的神经免疫和神经炎症过程针对神经特异性抗原。未被识别和因此未经治疗的PNDs常常致命，因此早期发现和积极治疗至关重要。虽然临床症状和体征的组合、详细的身体和神经影像学、临床神经生理学和电生理学研究以及肿瘤和神经系统组织活检分析都非常重要，但PNDs的确诊还依赖于在血液和/或脊髓脑脊液中发现相应的神经特异性旁肿瘤自身抗体。版权所有©2023 Elsevier B.V. All rights reserved.

Paraneoplastic neurological disorders (PNDs) are heterogeneous clinicopathologic syndromes that occur throughout the neuraxis resulting from damage to organs or tissues remote from the site of a malignant neoplasm or its metastases. The discordance between severe neurological disability and even an indolent malignancy suggests an underlying neuroimmunologic host immune response that inflicts nervous tissue damage while inhibiting malignant tumor growth. Motor system involvement, like other symptoms and signs, is associated with focal or diffuse involvement of the brain, spinal cord, peripheral nerve, neuromuscular junction or muscle, alone or in combination due to an underlying neuroimmune and neuroinflammatory process targeting neural-specific antigens. Unrecognized and therefore untreated, PNDs are often lethal making early detection and aggressive treatment of paramount importance. While the combination of clinical symptoms and signs, and analysis of detailed body and neuroimaging, clinical neurophysiology and electrodiagnostic studies, and tumor and nervous system tissue biopsies are all vitally important, the certain diagnosis of a PND rests with the discovery of a corresponding neural-specific paraneoplastic autoantibody in the blood and/or spinal cerebrospinal fluid.Copyright © 2023 Elsevier B.V. All rights reserved.