可逆性后部白质脑病综合征的临床及影像学特点
作者:赵伟秦,王得新,张慧 作者单位:100050首都医科大学附属北京友谊医院神经内科 《临床神经病学杂志》2007年10月20卷5期 论著
【摘要】 目的 探讨可逆性后部白质脑病综合征(rples)的临床和影像学特点。方法 回顾性分析5例rples患者的临床资料。结果 本组患者的原发病为:高血压1例,尿毒症3例,红斑狼疮1例。5例均为突然发作的血压增高、头痛伴呕吐、意识障碍及癫疒间等,3例有视觉障碍,肢体运动功能均正常。实验室检查5例均有肾功能不全。头颅ct或mri检查5例均显示双侧顶枕叶白质为主的较大范围的异常,其中ct为低密度、mri为长t1、长t2信号。经过积极的降压、脱水等治疗,4例患者临床症状及体征在短时间内即恢复良好,影像学复查也显示病变明显减轻或消失;1例死于脑疝。结论 rples的临床特点为在血压突然升高或使用某些药物的情况下急性出现的脑损害症状。发病机制可能与脑血管自动调节功能障碍有关。影像学特点为双侧、对称性后部脑白质较大范围的病变。大多数患者经治疗可很快恢复,极少数病情严重者也可导致死亡。
【关键词】 白质脑病;可逆性;临床;影像学
clinical and imaging features of reversible posterior leukoencephalopathy syndrome zhao weiqin, wang dexin,zhang hui. department of neurology, beijing friendship hospital,capital medical university, beijing 100050, china
abstract:objective to explore the clinical and imaging features of reversible posterior leukoencephalopathy syndrome (rples). methods the data of clinic five cases with rples were analyzed retrospectively.results the primary diseases in this group were hypertension for one case, uraemia for three cases and lupus nephropathy for another. clinical features of five patients include acute onset of hypertension,headache with vomiting. neurological symptoms were conscious disturbance, seizures (in 5 cases) and visual disorder (in 3 cases).the movement of limbs were normal. the abnormal laboratery data was renal inadequacy in 5 cases. the findings of imaging of all cases included bilateral white matter of parietal and occipital lobe abnormalities, which appeared as large areas of low density on ct scan, hypointense in t1 and hyperintense in t2 on mri scan. four cases presented clinical symptoms improvement in a short time and radiological recovery obviously after antihypertensive and anhydration therapy. one patient died caused on brain herniation.conclusions clinical features of rples is brain damage associated with malignant hypertension or the use of immunosuppressive agents. the most important pathogenesis is considered to be failure of the self regulation of cerebral vascular circulation . the imaging findings are bilateral symmetry cortical and subcortical edema in the posterior areas of the brain.most patients can be recovery by timely therapy, otherwise the irreversible neuronal injury even death can be also found in very few serious cases.
key words:leukoencephalopathy; reversible; clinic; imaging
可逆性后部白质脑病综合征(rples)又名可逆性大脑后部脑病综合征和可逆性后部脑水肿综合征[1],或高灌注脑病,1996年由hinchey等[2]首次提出,以后陆续有许多报道。现对我院近期收治的5例rples患者的临床和影像学特点分析如下。
1 临床资料
1.1 一般资料 本组男3例,女2例;年龄13~78岁,平均52岁;病程2~5 d,平均3.0 d。原发病为高血压1例、尿毒症3例和红斑狼疮(sle)1例。
1.2 临床表现 急性起病3例,亚急性起病2例。首发症状:头痛3例,头晕2例,均有呕吐。5例均有癫疒间发作及意识障碍(谵妄4例,昏迷1例),3例出现视觉障碍(包括视物不清、视物变形及偏盲)。肢体运动功能均正常。5例均出现血压增高和肾功能不全。
1.3 实验室检查 5例患者均行肾功能检查,尿素氮(bun)55~87.5 mg/dl,肌酐6~11.63 μg/dl。其他血液生化检查正常。
1.4 影像学检查 mri检查:4例显示双侧顶枕叶、2例显示颞叶较大范围的长t1、长t2信号,flair成像显示高信号。1例双侧小脑、脑干及丘脑也有同样改变。1例ct显示双侧顶枕叶低密度病灶。5例患者的病变均以皮质下为主,或皮质及皮质下均受累,并且多呈对称性(4例)。病灶酷似梗死,其中2例在较大的异常信号范围内出现小灶性出血。4例mri增强扫描均未见强化。3例病后2~4周复查头颅ct或mri,显示原异常部位病灶(ct低密度或mri长t1、长t2病灶)大部分消失或减轻。见图1、图2。
2 讨 论
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