A reversible posterior leukoencephalopathy syndrome

A reversible posterior leukoencephalopathy syndrome
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DOI:
10.1056/nejm199602223340803
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发表时间:
1996-02-22
影响因子:
158.5
通讯作者:
Caplan, LR
Caplan, LR
中科院分区:
医学1区
文献类型:
--
作者:
Hinchey, J;Chaves, C;Caplan, LR

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研究背景与方法。在一些因急性疾病住院的患者中,我们注意到一种可逆的头痛综合征、精神功能改变、癫痫发作和视力丧失,这些症状在影像学检查中主要显示为后脑白质病。为了阐明这种综合征,我们检索了记录在波士顿新英格兰医学中心和巴黎圣安妮医院进行的计算机断层扫描(CT)和磁共振成像(MRI)研究的日志簿;从1988年到1994年,我们发现了15例这样的患者。15例患者中,7例在移植后接受免疫抑制治疗或再生障碍性贫血治疗,1例因黑色素瘤接受干扰素治疗,3例子痫,4例急性高血压性脑病合并肾脏疾病(狼疮性肾炎2例,急性肾小球肾炎1例,对乙酰氨基酚所致肝肾衰竭1例),12例血压突然升高,8例肾功能有一定损害。临床表现包括头痛、呕吐、精神混乱、癫痫、皮质性失明和其他视觉异常,以及运动体征。CT和MRI研究显示广泛的双侧白质异常,提示大脑半球后部区域水肿,但这些变化通常涉及其他大脑区域、脑干或小脑。患者均给予抗高血压药物治疗,并停止或减少免疫抑制治疗,15例患者神经功能缺损均在2周内消退。可逆的,以后部白质脑病为主,可发生在肾功能不全、高血压或免疫抑制的患者中。神经影像学表现为皮质下水肿,无梗死。(C) 1996年,马萨诸塞州医学会。
Background and Methods. In some patients who are hospitalized for acute illness, we have noted a reversible syndrome of headache, altered mental functioning, seizures, and loss of vision associated with findings indicating predominantly posterior leukoencephalopathy on imaging studies, To elucidate this syndrome, we searched the log books listing computed tomographic (CT) and magnetic resonance imaging (MRI) studies performed at the New England Medical Center in Boston and Hopital Sainte Anne in Paris; we found 15 such patients who were evaluated from 1988 through 1994.Results. Of the 15 patients, 7 were receiving immunosuppressive therapy after transplantation or as treatment for aplastic anemia, 1 was receiving interferon for melanoma, 3 had eclampsia, and 4 had acute hypertensive encephalopathy associated with renal disease (2 with lupus nephritis, 1 with acute glomerulonephritis, and 1 with acetaminophen-induced hepatorenal failure), Altogether, 12 patients had abrupt increases in blood pressure, and 8 had some impairment of renal function. The clinical findings included headaches, vomiting, confusion, seizures, cortical blindness and other visual abnormalities, and motor signs, CT and MRI studies showed extensive bilateral white-matter abnormalities suggestive of edema in the posterior regions of the cerebral hemispheres, but the changes often involved other cerebral areas, the brain stem, or the cerebellum. The patients were treated with antihypertensive medications, and immunosuppressive therapy was withdrawn or the dose was reduced, In all 15 patients, the neurologic deficits resolved within two weeks.Conclusions. Reversible, predominantly posterior leukoencephalopathy may develop in patients who have renal insufficiency or hypertension or who are immunosuppressed, The findings on neuroimaging are characteristic of subcortical edema without infarction. (C) 1996, Massachusetts Medical Society.