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[可逆性后部白质脑病综合征脑干变异型伴临床病程延长:一例报告]

[Brainstem variant of reversible posterior leukoencephalopathy syndrome with a prolonged clinical course: a case report].

作者信息

Oishi Chizuko, Okano Haruko, Uegama Kazuya, Kobayashi Keiichi, Nagane Motoo, Chiba Atsuro, Sakuta Manabu

机构信息

Department of Neurology, Kyorin University School of Medicine.

出版信息

Rinsho Shinkeigaku. 2008 Oct;48(10):737-41. doi: 10.5692/clinicalneurol.48.737.

Abstract

A 38-year-old man gradually developed gait instability, dysarthria, and dysphagia over two months associated with an elevated blood pressure after starting hemodialysis therapy for diabetic nephropathy. Brain MRI studies indicated vasogenic edema in the brainstem, extending from the lower midbrain to the upper medulla oblongata. The patient's high blood pressure was refractory to treatment, and his neurological disabilities and MRI abnormalities progressed. FDG-PET, MR spectroscopy, and cerebrospinal fluid studies did not suggest neoplastic pathologies. The patient was diagnosed with a brainstem variant of reversible posterior leukoencephalopathy syndrome, and received three courses of steroid pulse therapy. After the pulse therapy, the clinical manifestations and MR findings improved. By maintaining strict management of blood pressure and body water balance during hemodialysis, he did not experience any further clinical exacerbation, and the lesion on MR images continued to regress. Ten months after the pulse therapy, T1-weighted images showed slightly hyperintense signal. This case suggests that reversible posterior leukoencephalopathy syndrome (RPLS) may take a chronic clinical course without acute onset.

摘要

一名38岁男性在开始接受糖尿病肾病血液透析治疗后两个月内逐渐出现步态不稳、构音障碍和吞咽困难,并伴有血压升高。脑部MRI研究显示脑干存在血管源性水肿,从中脑下部延伸至延髓上部。患者的高血压对治疗无效,其神经功能障碍和MRI异常情况不断进展。FDG-PET、磁共振波谱分析和脑脊液研究均未提示肿瘤性病变。该患者被诊断为可逆性后部白质脑病综合征的脑干变异型,并接受了三个疗程的类固醇脉冲治疗。脉冲治疗后,临床表现和MR表现均有所改善。通过在血液透析期间严格控制血压和身体水平衡,他未再出现任何临床症状加重的情况,MR图像上的病变也持续消退。脉冲治疗十个月后,T1加权图像显示信号略高。该病例表明,可逆性后部白质脑病综合征(RPLS)可能呈现慢性临床病程,而非急性起病。

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