Hsam Nang Boe Ohnmar, Angstwurm Klemens, Peters Sebastian, Fuchs Kornelius, Schuierer Gerhard, Bogdahn Ulrich, Weissert Robert
Department of Neurology, University of Regensburg, Regensburg, Germany.
Front Neurol. 2017 Jul 27;8:345. doi: 10.3389/fneur.2017.00345. eCollection 2017.
We describe an 18-year-old patient who developed back pain, rapidly ascending sensomotory deficits, bladder dysfunction, Lhermitte's sign, absent abdominal reflexes of all three levels, brisk tendon reflexes, and positive Babinski's sign. Magnetic resonance imaging of the spinal cord showed a long segment of cervical and thoracic intramedullary signal hyperintensity suggesting a longitudinally extensive transverse myelitis possibly within the course of a fast progressing ascending immune-mediated hemorrhagic myelopathy. Throughout his illness, the patient deteriorated with tetraplegia, cardiac arrest, and respiratory failure although he received, after exclusion of infective causes, therapy with steroids, immunoglobulins, plasmapheresis, and cyclophosphamide. Interestingly, treatment with the C5-inhibitor eculizumab to prevent complement-mediated spinal cord injury achieved an arrest of clinical deterioration. We propose eculizumab as treatment in fast progressive and potentially fatal immune-mediated spinal cord injury. Furthermore, this case raises awareness for the process of clinical decision-making in severe myelopathies.
我们描述了一名18岁患者，该患者出现背痛、快速进展的感觉运动功能缺损、膀胱功能障碍、莱尔米特征（Lhermitte's sign）、所有三个节段的腹壁反射消失、腱反射亢进以及巴宾斯基征阳性。脊髓磁共振成像显示颈段和胸段脊髓长节段髓内信号高，提示可能为快速进展的上升性免疫介导出血性脊髓病过程中的纵向广泛横贯性脊髓炎。在整个病程中，尽管在排除感染性病因后，患者接受了类固醇、免疫球蛋白、血浆置换和环磷酰胺治疗，但仍出现四肢瘫痪、心脏骤停和呼吸衰竭，病情恶化。有趣的是，使用C5抑制剂依库珠单抗治疗以预防补体介导的脊髓损伤使临床病情恶化得到了控制。我们建议将依库珠单抗用于快速进展且可能致命的免疫介导脊髓损伤的治疗。此外，该病例提高了对严重脊髓病临床决策过程的认识。
