Department of Neurology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China.
BMC Neurol. 2023 Oct 4;23(1):352. doi: 10.1186/s12883-023-03383-4.
Primary intramedullary spinal cord lymphoma (PISCL) is rare and easily misdiagnosed with the lack of typical clinical features and non-specific imaging manifestations.
A 49-year-old man was admitted to our hospital because of persistent limbs numbness, pinprick-like pain in the posterior neck and unsteady gaits. He has brisk tendon reflexes and positive Babinski's sign. Magnetic resonance imaging (MRI) of the cervical spine showed an abnormal signal with aberrant reinforcement at medulla oblongata and the level of C1-C7. He was clinically diagnosed as longitudinally extensive transverse myelitis (antibody-negative). Steroid pulse therapy was administered and resulted in reduced symptoms. One month later, his situation was exacerbated compared to the onset. We launched a new cascade of steroid pulse therapy. But it did not improve his symptoms. Finally, the biopsy pathology confirmed PISCL. Chemotherapy, radiotherapy and zanubrutinib were administered and until now about 3 years into treatment the patient is still survival.
Based on our case and literature review, we recommend that spinal onset patients react ineffectively to standard immunoglobulins or hormonal treatments or experience a relapse after a short time relief should take PISCL into consideration.
原发性脊髓内淋巴瘤（PISCL）较为罕见，由于缺乏典型的临床特征和非特异性的影像学表现，容易误诊。
一名 49 岁男性因四肢麻木、颈后刺痛样疼痛和步态不稳而入院。他的腱反射活跃，巴氏征阳性。颈椎磁共振成像（MRI）显示延髓和 C1-C7 水平异常信号伴异常强化。临床上诊断为广泛横贯性脊髓炎（抗体阴性）。给予类固醇脉冲治疗后症状减轻。一个月后，他的病情比发病时恶化。我们开始了新一轮的类固醇脉冲治疗，但并未改善他的症状。最终，活检病理证实为 PISCL。给予化疗、放疗和泽布替尼治疗，截至目前，治疗已进行约 3 年，患者仍存活。
基于我们的病例和文献复习，我们建议对于脊髓起病、对标准免疫球蛋白或激素治疗反应不佳或在短暂缓解后复发的患者，应考虑 PISCL。
