可逆性后部白质脑病综合征的临床和影像学特征：9例回顾性病例系列研究

Clinical and hnaghtg features of reversible posterior leukoencepha- lopathy syndrome： a retrospective study of 9 cases

目的探讨可逆性后部白质脑病综合征（reversible posterior lettkoencephalopathy syndrome，RPLS）的临床和影像学特征。方法回顾性分析9例RPLS患者的临床和影像学资料。结果继发于妊娠高血压综合征4例（44％），短肠综合征1例（11％），急性淋巴细胞白血病1例（11％），IgA肾病1例（11％），高血压脑病2例（22％），平均发病年龄（23．4±9．9）岁。临床主要表现为头痛、癫痫发作、视物模糊和意识障碍。MR／均显示为双侧大脑后部白质对称性病灶，T1加权成像和弥散加权成像呈低或等信号，T2加权成像、液体衰减翻转恢复序列以及表观弥散系数图呈高信号。经治疗后患者症状消失，MRI复查病灶完全或大部分消失。结论RPLS病因各异，影像学主要表现为大脑后部白质血管源性脑水肿信号，及时治疗后症状和影像学特征迅速改善，发病年龄较轻是其重要的临床特点。

Objective To investigate the clinical and imaging features of the reversible posterior leukoencephalopathy syndrome （RPLS）. Methods The clinical and imaging data of 9 patients with RPLS were analyzed retrospectively. Results Among the patients, 4 （44%） were secondary to pregnancy-induced hypertension syndrome, 1 （11% ） was second to short bowel syndrome, 1（11% ） was second to acute lymphoblastic leukemia, 1 （ 11% ） was second to IgA nephropathy, and 2 （22%） were second to hypertensive encephaiopathy. Their mean age of symptom onset was 23.4 ＋-9. 9 years. Their main clinical manifestations were headaches, seizures, blurred vision, and consciousness disturbance. Magnetic resonance imaging （MRI） showed symn^trical lesions of bilatered white matter in posterior brain. T1 weighted imat^g and diffusion-weighted imaging showed low or isointense signal. T2 weighted imaging, fluid attenuated inversion recovery （FLAIR）, and apparent diffusion coefficient （ADC） map showed high-intensi- ty signal. The symptoms of the patients disappeared after the treatment. MR/ reexamination showed that the lesions disappeared completely or almost completely. Conclusions The causes of RPLS were different. The imaging mainly showed the signal of vasogenic brain edema on the posterior white matter of the brain. After timely treatment, the symptoms and imaging features were improved rapidly. The patients with young-onset RPLS are their important clinical feature.