脑后部可逆性脑病综合征临床特征分析

Clinical spectrum of posterior reversible encephalopathy syndrome

目的通过探讨脑后部可逆性脑病综合征(PRES)临床表现及影像学特征,总结其诊断及治疗策略。方法回顾性分析具有特征性PRES临床及影像表现患者的相关病因、临床表现及头颅磁共振成像(MRI)、脑电图(EEG)、脑脊液检查结果。结果共发现9例PRES患者(男1例,女8例)。相关病因分别为:原发性高血压、妊娠相关、甲强龙治疗、肝硬化及系统性红斑狼疮。临床表现包括头痛、呕吐、痫性发作、意识障碍、视觉障碍、精神异常或视幻觉。发病期间的最高收缩压平均为161.7mm Hg。其临床症状康复时间多数患者在7 d之内。头颅MRI显示大部分患者有不典型表现,病灶累及额叶、颞叶、小脑、脑干、丘脑及基底节区。未见出血或单侧病灶。EEG显示为慢波。结论头颅MRI是诊断该病的金标准,但其中不典型影像表现较为多见。若及早诊断、治疗,该病预后较好。

Objective To retrospectively identify characteristic clinical presentations and neuroimaging abnormalities in patients with posterior reversible encephalopathy syndrome（PRES） and to summarize the diagnosis and treatment strategy.Methods To retrospectively analyze associated comorbid medical conditions,presenting clinical symptoms,diagnostic test results（magnetic resonance imaging,electroencephalography,and lumbar puncture）,and time to clinical recovery in the patients with PRES.Results 9 patients with PRES（8 females and 1 male） were identified with a mean age of 46.3 years.Comorbid conditions included hypertension,eclampsia,teroid therapy,cirrhosis and systemic lupus erythematosus.Presenting symptoms included headache,vomiting,seizures,altered mental status,visual impairment,and psychiatric symptoms or visual hallucination.Mean peak systolic blood pressure at presentation was 161.7 mm Hg.Clinical symptoms resolved after a mean of 5.3 d in most patients.Atypical neuroimaging features included frontal,temporal,cerebellum,brainstem,thalamus and basal ganglia involvement in most patients,unilateral lesions or hemorrhage were not found.Electroencephalogram demonstrated slow wave activity.Conclusion Clinical recovery occurres in most patients within 1 week.MRI is the golden standard of diagnosing this entity.Atypical neuroimaging features were frequent.Early diagnosis and proper treatment is essential in the management of PRES.