以累及脑干为主的可逆性后部脑病综合征6例临床、影像学分析

Brainstem-Dominant Posterior Reversible Encephalopathy Syndrome: Clinical and Radiologic Findings of 6 Cases

目的探讨以累及脑干为主的可逆性后部脑病综合征(posterior reversible encephalopathy syndrome,PRES)的临床特征及影像学表现,研究其发病机制。资料与方法回顾性分析6例以累及脑干为主的PRES患者的临床和影像学资料。6例中5例为原发性高血压患者,1例为慢性肾功能不全患者。临床主要表现为头晕、头痛及肢体活动障碍,无明显抽搐发作、视物障碍及神经系统局灶性定位体征。结果 MRI表现为脑干内异常信号,多累及延髓、小脑,也可合并幕上室旁白质病变。对症治疗后症状明显改善、影像学恢复正常提示其良性疾病过程。结论脑干型PRES作为一种影像学分布模式具有独特的临床表现形式。MRI对本病的诊断和鉴别诊断具有重要价值。

Objective To investigate the clinical teatures and radiologic characteristics of brainstem-dominant posterior reversible encephalopathy syndrome（PRES） and furthermore, to investigate its pathogenesis. Materials and Methods The clinical and radiologic data of six patients with brainstem-dominant PRES were retrospectively analyzed. Five cases had primary hypertensive history and one had chronic renal insufficiency. Results All cases presented with headache, dizzi- ness and limbs weakness without seizures, visual disorder, focal neurologic signs and symptoms. MRI showed slightly hypo- intensity on T1 -weighted imaging and hyper-intensity on T2-weighted imaging in brainstem with dominantly medulla oblonga- ta and cerebellum affected, occasionally in periventrieular white matter regions. Marked improvement and completely resolu- tion on radiologic follow-up after treatment implied a benign process for the entity. Conclusion Brainstem- dominant PRES, as a radiologic pattern, has its distinctive clinical symptoms different from typical PRES patients. MRI is crucial to the diagnosis and differential diagnosis for this not uncommon entity.