可逆性后部白质脑病综合征13例临床及影像学分析

Clinical and imaging analysis of 13 patients with reversible posterior leukoencephalopathy syndrome

目的探讨可逆性后部白质脑病综合征(RPLS)的病因、病理机制、临床及影像学特点,提高临床医师对RPLS的认识,减少漏诊和误诊。方法回顾性分析13例RPLS患者的临床资料。结果本组患者的原发病为:妊高征子痫或子痫前期6例,嗜铬细胞瘤1例,IgA肾病1例,慢性肾炎伴肾功能不全1例,系统性红斑狼疮3例,吸食"K粉"1例。发病时血压多升高,主要临床表现为头痛、癫痫发作、视觉障碍、不同程度意识障碍。神经影像学典型改变是大脑后部、皮质下为主的、双侧对称性、斑片状的血管源性水肿。一般预后较好,死亡1例。结论 RPLS的病因、发病机制并不相同,早诊断、早治疗很重要,控制血压和终止癫痫发作是治疗的关键。

Objective To explore the etiologies, pathophysiology, clinical and imaging features, diagnosis and differential diagnosis, therapy and prognosis of reversible posterior leukoencephalopathy syndrome （RPLS）, so as to raise the doctor＇s attention to RPLS, and reduce missed diagnosis and avoid misdiagnosis. Methods The clinical and imaging data of 13 cases of RPLS were analyzed retrospectively. Results The primary medical conditions in this group were edarnpsia or preeclampsia of pregnancy-induced hypertension for 6 cases, pheochromocytoma for 1 case, IgA nephropathy for 1 case, chronic nephritis with renal insufficiency for 1 case, systemic lupus erythematosus （SLE） for 3 cases, pheochromocytoma for 1 cases and inhalation of ketamine for 1 case. In acute onset, RPLS mainly presented as seizures, headache, altered mental stations, visual disturbance, and most of the patients had an abrupt and significant elevation in blood pressure. The characterized neuroimaging features of RPLS were associated with subcortical vasogenic edema, patchy symmetrical bilateral involvement with preferential involvement of the posterior head regions, The majority of patients with RPLS had an excellent prognosis. Conclusions The etiological factors and pathogenesis of RPLS are different and not all of them are reversible. Thus early diagnosis and early treatment is important. The control of blood pressure and termination of seizures are the key points of treatment.