Department of Neurosurgery, Tianjin Medical University General Hospital, 154 Anshan Road, Tianjin, 300052, China.
Tianjin Neurological Institute, Key Laboratory of Post-Trauma Neuro-Repair and Regeneration in Central Nervous System, Ministry of Education & Key Laboratory of Injuries, Variations and Regeneration of Nervous System, 154 Anshan Road, Tianjin, 300052, China.
Neurol Sci. 2024 Apr;45(4):1447-1454. doi: 10.1007/s10072-023-07192-y. Epub 2023 Nov 22.
Neurolymphomatosis (NL) is an uncommon malignant lymphoma characterized by selective infiltration of the central and peripheral nervous system. In this case report, we present a patient diagnosed with diffuse large B-cell lymphoma who initially manifested with peripheral neuropathy, primarily characterized by weakness of the left lower limb. By exploring its clinical manifestations, ancillary tests, and reviewing the relevant literature, we aim to deepen our understanding, diagnosis, and treatment of this disease. A 48-year-old male patient presented to the Department of Neurology, Hematology, and Neurosurgery with complaint of left lower limb weakness that had persisted for over 11 months. Initial laboratory tests and cerebrospinal fluid analysis yielded negative results. Electromyography examination indicated damage to the left lumbar plexus and iliac plexus nerves raising suspicions of nerve root involvement. Enhanced MRI of the lumbosacral plexus nerves revealed thickening and enhanced signals in left nerve roots at T12-L1, L1-2, and L3-4 levels. Additionally, local thickening and enhancement of signals were observed in the left erector spine muscle, psoas major, and iliopsoas muscles compared to the contralateral side. PEC/CT imaging displayed multiple soft tissue density shadows in the left foraminal area at the T12-1 and L1-2 levels. Bone marrow examination excluded hematological disease. Subsequent biopsy of the left foraminal nerve root at T12-L1 and the vertebral muscle at L3 level confirmed a diagnosis of diffuse large B-cell malignant lymphoma, indicating PNSL due to the involvement of multiple nerve roots. Following diagnosis, the patient underwent chemotherapy, resulting in the alleviation of his symptoms. Diagnosing PNSL can be challenging due to the nonspecific clinical manifestations and often inconclusive laboratory test results. Misdiagnosis and delayed diagnosis are common pitfalls. Electromyography may reveal damage to the affected peripheral nerves, while MR imaging might show nerve root thickening, and PET/CT can demonstrate increased lesion uptake. However, the definitive diagnosis relies on a biopsy of the lesion. Treatment for PNSL typically involves chemotherapy.
神经淋巴瘤病（NL）是一种罕见的恶性淋巴瘤，其特征为中枢和周围神经系统的选择性浸润。在本病例报告中，我们介绍了一位诊断为弥漫性大 B 细胞淋巴瘤的患者，其最初表现为周围神经病，主要表现为左下肢无力。通过探讨其临床表现、辅助检查，并复习相关文献，旨在加深对该病的认识、诊断和治疗。
一位 48 岁男性患者因左下肢无力持续 11 个月余就诊于神经内科、血液科和神经外科。初始实验室检查和脑脊液分析结果均为阴性。肌电图检查提示左侧腰骶丛和髂腰肌神经受损，提示神经根受累。腰骶丛神经增强 MRI 显示 T12-L1、L1-2 和 L3-4 水平左侧神经根增粗伴信号增强，左侧竖脊肌、腰大肌和髂腰肌与对侧相比局部增粗伴信号增强。PECT/CT 影像显示 T12-1 和 L1-2 左侧椎间孔区多个软组织密度影。骨髓检查排除血液系统疾病。随后行 T12-L1 左侧椎间孔神经根和 L3 椎体肌活检，明确诊断为弥漫性大 B 细胞恶性淋巴瘤，考虑为累及多根神经根的原发性神经外胚层肿瘤（PNSL）。诊断后，患者接受了化疗，症状得到缓解。
由于临床表现不特异且实验室检查结果常不明确，PNSL 的诊断具有挑战性。误诊和延迟诊断较为常见。肌电图可能显示受累外周神经损伤，磁共振成像（MRI）可能显示神经根增粗，而正电子发射断层扫描（PET）/CT 可能显示病变摄取增加。然而，明确诊断依赖于病变活检。PNSL 的治疗通常包括化疗。
