Akhvlediani R, Mogylevsky A, Contant S
Regional Central Hospital of Sens, France.
Georgian Med News. 2008 Jan(154):22-6.
The article describes the case history of posterior leukoencephalopathy syndrome - a brain disorder that predominantly affects the cerebral white matter. Edematous lesions involve the posterior parietal and occipital lobes, and may spread to basal ganglia, brain stem and cerebellum. This rapidly evolving neurological condition is clinically characterized by headache, nausea and vomiting, seizures, visual disturbances and altered sensorial functions, and occasionally focal neurological deficit. Posterior leukoencephalopathy syndrome is often associated with an abrupt increase in blood pressure and is usually seen in patients with eclampsia, renal disease and hypertensive encephalopathy. It is also seen in the patients treated with cytotoxic and immunosuppressive drugs such as cyclosporine A, tacrolimus /FK-506, cisplatin, cytarabine, IVIg, erytrophoietin, and interferon alpha. The study demonstrated that lesions of posterior leukoencephalopathy syndrome are best visualized with magnetic resonance [MR] imaging. T2 weighted MR Images, at the height of symptoms, characteristically show diffuse hyper intensity selectively involving the parieto - occipital white matter. Occasionally the lesions also involve the grey matter. Computed tomography can also be used satisfactorily to detect hypodense lesions of posterior leukoencephalopathy. Early recognition of this condition is of paramount importance because prompt control of blood pressure, withdrawal of immunosuppressive agents will cause reversal of the syndrome. Delay in the diagnosis and treatment can result in permanent damage to affected brain tissues. The clinical data and radiological findings depicted in the study add to the investigation of the disorder.
本文描述了后部白质脑病综合征的病例史——一种主要影响脑白质的脑部疾病。水肿性病变累及顶叶后部和枕叶,可蔓延至基底神经节、脑干和小脑。这种迅速发展的神经系统疾病的临床特征为头痛、恶心、呕吐、癫痫发作、视觉障碍和感觉功能改变,偶尔还会出现局灶性神经功能缺损。后部白质脑病综合征常与血压突然升高有关,通常见于子痫、肾病和高血压脑病患者。在用细胞毒性和免疫抑制药物(如环孢素A、他克莫司/FK-506、顺铂、阿糖胞苷、静脉注射免疫球蛋白、促红细胞生成素和干扰素α)治疗的患者中也可见到。该研究表明,磁共振成像(MR)能最好地显示后部白质脑病综合征的病变。在症状最严重时的T2加权磁共振图像特征性地显示弥漫性高信号,选择性累及顶枕白质。偶尔病变也累及灰质。计算机断层扫描也可令人满意地用于检测后部白质脑病的低密度病变。早期识别这种疾病至关重要,因为迅速控制血压、停用免疫抑制剂将使综合征逆转。诊断和治疗延迟可导致受影响脑组织的永久性损伤。该研究中描述的临床数据和影像学发现为该疾病的研究增添了内容。