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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 investigate the etiology, pathology, clinical and imaging features of reversible posterior leukoencephalopathy syndrome (RPLS) and to improve clinicians’ understanding of RPLS and to reduce misdiagnosis and missed diagnosis. Methods The clinical data of 13 patients with RPLS were retrospectively analyzed. Results The primary disease in this group was 6 cases of PIH or preeclampsia, 1 case of pheochromocytoma, 1 case of IgA nephropathy, 1 case of chronic nephritis with renal insufficiency, 3 cases of systemic lupus erythematosus, “K powder ” in 1 case. When the onset of increased blood pressure, the main clinical manifestations of headache, seizures, visual disorders, varying degrees of disturbance of consciousness. Typical changes in neuroimaging are posterior cerebral cortex, predominantly bilateral, symmetrical, patchy vasogenic edema. The general prognosis is good, 1 patient died. Conclusion The etiology and pathogenesis of RPLS are not the same. Early diagnosis and early treatment are very important. Controlling blood pressure and terminating seizure is the key to treatment.