Maeda Yasuhiro, Watanabe Mitsuru, Maeda Norihisa, Ogata Hidenori, Shinoda Koji, Iwaki Toru, Kira Jun-Ichi
Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University.
Department of Neuropathology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University.
Rinsho Shinkeigaku. 2020 May 26;60(5):351-357. doi: 10.5692/clinicalneurol.cn-001413. Epub 2020 Apr 18.
The patient was a 40-year-old woman who was previously diagnosed with systemic lupus erythematosus and myasthenia gravis and had received prednisolone and tacrolimus for more than 7 years. In February 2017, she noticed pain in her lower back and weakness of the lower limbs, and was referred to our hospital on day 5. She had shingles in the right lower thoracic dermatomes and Brown-Séquard syndrome with right-sided dominant weakness in her lower limbs and left-sided superficial sensory disturbance below the L1 level. Varicella zoster virus (VZV)-associated myelopathy was suspected because of her symptoms and clinical findings. Despite the immediate administration of intravenous acyclovir after hospitalization, she lost consciousness and experienced a seizure related to cerebral hemorrhage in the left temporal lobe on the night of day 5. MRI showed enhanced lesions along the spinal cord and leptomeninges of the brainstem and temporal lobe. VZV-IgG and VZV-DNA were positive in the cerebrospinal fluid. Based on these clinical features and laboratory findings, she was diagnosed as VZV-associated vasculopathy and myelopathy. She subsequently had multiple cerebral infractions and hemorrhage, and developed sudden cardiopulmonary arrest on day 6, culminating in death on day 17. Autopsy showed that inflammatory mononuclear cells had infiltrated the vascular walls of the spinal cord. Immunohistochemistry revealed that some neurons and macrophages in the white matter of the spinal cord were positive for VZV. In addition, atrophic neurons, satellite cells surrounding these neurons, and infiltrating macrophages were immune-positive for VZV at the L2 dorsal root ganglia. These findings were consistent with VZV-associated vasculopathy and myelitis. Under immunosuppressive conditions, VZV can cause shingles and neuronal complications such as vasculopathy and myelitis, which are sometimes fatal despite the immediate administration of intravenous acyclovir. New treatment drugs or drugs to prevent VZV activation are desired.
该患者为一名40岁女性，既往诊断为系统性红斑狼疮和重症肌无力，接受泼尼松龙和他克莫司治疗超过7年。2017年2月，她注意到下背部疼痛和下肢无力，于第5天转诊至我院。她在右下胸皮节出现带状疱疹，并伴有布朗 - 塞卡尔综合征，下肢右侧为主的无力以及L1水平以下左侧浅感觉障碍。由于她的症状和临床表现，怀疑为水痘 - 带状疱疹病毒（VZV）相关脊髓病。住院后尽管立即给予静脉注射阿昔洛韦，但她在第5天夜间失去意识并出现与左颞叶脑出血相关的癫痫发作。MRI显示脊髓、脑干和颞叶软脑膜有强化病变。脑脊液中VZV - IgG和VZV - DNA呈阳性。基于这些临床特征和实验室检查结果，她被诊断为VZV相关血管病和脊髓病。随后她出现多处脑梗死和出血，并在第6天突发心肺骤停，最终于第17天死亡。尸检显示炎症单核细胞浸润脊髓血管壁。免疫组织化学显示脊髓白质中的一些神经元和巨噬细胞VZV呈阳性。此外，L2背根神经节处萎缩的神经元、围绕这些神经元的卫星细胞以及浸润的巨噬细胞VZV免疫阳性。这些发现与VZV相关血管病和脊髓炎一致。在免疫抑制状态下，VZV可引起带状疱疹以及血管病和脊髓炎等神经元并发症，尽管立即给予静脉注射阿昔洛韦，有时仍会致命。需要新的治疗药物或预防VZV激活的药物。
