Progressive multifocal leukoencephalopathy resembling central nervous system systemic lupus erythematosus.
Progressive multifocal leukoencephalopathy resembling central nervous system systemic lupus erythematosus.
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进行性多灶性白质脑病，类似于中枢神经系统系统性红斑狼疮。
DOI：
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发表时间：
1998
影响因子：
3.7
通讯作者：
M. Sumiya
中科院分区：
文献类型：
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作者：
M. Kinoshita;K. Iwana;H. Shinoura;S. Aotsuka;M. Sumiya
一名 21 岁女性，有 6 年 SLE 病史，出现言语障碍、右手笨拙以及活动性疾病表现，提示中枢神经系统 SLE。尽管对系统性红斑狼疮进行了积极的治疗，但她的神经系统状况仍然恶化。 MRI 显示双侧大脑和小脑白质 T1 加权图像低强度，T2 加权图像高强度，与进行性多灶性白质脑病 (PML) 相符。椎管内注射干扰素-β 似乎可以减缓她的 MRI 和神经系统检查结果的恶化。然而，她最终出现了去大脑强直，并因念珠菌血症而死亡。通过聚合酶链式反应技术在尸检大脑中检测到 JC 病毒 DNA。在检查患有神经系统异常的 SLE 患者时，应始终牢记 PML。
A 21-year-old woman with a 6-year history of SLE presented with a speech disturbance and right hand clumsiness along with manifestations of active disease suggesting central nervous system SLE. Despite aggressive treatment for SLE, her neurological condition worsened. MRI demonstrated low intensity in T1-weighted images and high intensity in T2-weighted images in the white matter of the bilateral cerebrum and cerebellum, compatible with progressive multifocal leukoencephalopathy (PML). Intraspinal administration of interferon-beta seemed to slow the deterioration of her MRI and neurological findings. However, she eventually developed decerebrate rigidity and died due to candidemia. DNA of the JC virus was detected in the autopsied brain by the polymerase chain reaction technique. PML should always be borne in mind when examining patients with SLE showing neurological abnormalities.