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可逆性后部白质脑病综合征非典型脑干变异型的影像学和病理学表现

Radiographic and pathologic findings in an atypical brainstem variant of reversible posterior leukoencephalopathy syndrome.

作者信息

Decker David A, Falchook Adam D, Yachnis Anthony T, Waters Michael F

机构信息

Department of Neurology, University of Florida College of Medicine, Gainesville, FL 32610, USA.

出版信息

Neurologist. 2009 Nov;15(6):364-6. doi: 10.1097/NRL.0b013e3181951ac7.

Abstract

BACKGROUND

Reversible posterior leukoencephalopathy syndrome (RPLS) and hypertensive encephalopathy (HE) are terms generally used interchangeably to describe a syndrome characterized by encephalopathy, focal deficits, and vasogenic edema seen on magnetic resonance imaging, which are potentially reversible with treatment. The underlying pathologic changes are less well defined. Previously, the only pathologic data available came from a single autopsy series. Results from a recent biopsy report differ with the autopsy series leading to the suggestion that RPLS and HE may be distinct.

CASE REPORT

We report a markedly hypertensive patient with encephalopathy and hemiparesis and focal edema in the brainstem visualized on magnetic resonance imaging. A biopsy was performed that demonstrated pathologic changes associated with RPLS. With treatment of hypertension, the patient's symptoms resolved completely.

CONCLUSIONS

We report an unusual brainstem variant of RPLS, adding to the neuropathologic features of this syndrome, and supporting the predominant view that RPLS and HE have a shared pathologic basis.

摘要

背景

可逆性后部白质脑病综合征(RPLS)和高血压脑病(HE)通常可互换使用,用于描述一种以脑病、局灶性神经功能缺损以及磁共振成像上可见的血管源性水肿为特征的综合征,经治疗后这些症状可能可逆。其潜在的病理变化尚不明确。此前,仅有的病理数据来自单一尸检系列。最近一份活检报告的结果与尸检系列不同,这表明RPLS和HE可能是不同的。

病例报告

我们报告一例血压显著升高的患者,伴有脑病、偏瘫以及磁共振成像显示的脑干局灶性水肿。进行了活检,结果显示存在与RPLS相关的病理变化。经高血压治疗后,患者症状完全缓解。

结论

我们报告了RPLS一种不常见的脑干变异型,增加了该综合征的神经病理学特征,并支持RPLS和HE具有共同病理基础这一主流观点。

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