Hinchey J, Chaves C, Appignani B, Breen J, Pao L, Wang A, Pessin M S, Lamy C, Mas J L, Caplan L R
Department of Neurology, New England Medical Center, Boston, MA 02111, USA.
N Engl J Med. 1996 Feb 22;334(8):494-500. doi: 10.1056/NEJM199602223340803.
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 Hôpital Sainte Anne in Paris; we found 15 such patients who were evaluated from 1988 through 1994.
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 The patients were treated with antihypertensive medications, and immunosuppressive therapy was withdrawn or the dose was reduced.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.
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.
在一些因急性疾病住院的患者中,我们注意到一种可逆性综合征,其症状包括头痛、精神功能改变、癫痫发作和视力丧失,影像学检查显示主要为后部白质脑病。为阐明这一综合征,我们查阅了波士顿新英格兰医疗中心和巴黎圣安妮医院的计算机断层扫描(CT)和磁共振成像(MRI)检查记录;我们发现了15例在1988年至1994年期间接受评估的此类患者。
15例患者中,7例在移植后或作为再生障碍性贫血的治疗接受免疫抑制治疗,1例因黑色素瘤接受干扰素治疗,3例发生子痫,4例患有与肾病相关的急性高血压脑病(2例为狼疮性肾炎,1例为急性肾小球肾炎,1例为对乙酰氨基酚引起的肝肾衰竭)。共有12例患者血压突然升高,8例存在一定程度的肾功能损害。临床症状包括头痛、呕吐、意识模糊、癫痫发作、皮质盲及其他视觉异常,以及运动体征。CT和MRI检查显示广泛的双侧白质异常,提示大脑半球后部区域水肿,但病变常累及其他脑区、脑干或小脑。患者接受了抗高血压药物治疗,免疫抑制治疗被停用或剂量减少。所有15例患者的神经功能缺损在两周内均得到缓解。
肾功能不全、高血压或免疫抑制的患者可能发生可逆性、主要为后部的白质脑病。神经影像学表现为皮质下水肿而非梗死的特征。