肝豆状核变性患者的脑诱发电位和颅脑MRI表现分析  

Changes of brain evoked potential and brain MRI in 15 patients with Wilson's disease

作　　者：李文杰[1] 王晓平[2] 

机构地区：[1]上海交通大学附属第一人民医院神经内科,200080 [2]宁波市第一医院老年科

出　　处：《中华脑科疾病与康复杂志（电子版）》2013年第2期23-28,共6页Chinese Journal of Brain Diseases and Rehabilitation（Electronic Edition）

摘　　要：目的观察15例肝豆状核变性（WD）患者的脑诱发电位、颅脑MRI改变。方法对15例WD患者和15例健康对照者进行脑干听觉诱发电位（BAEP）、视觉诱发电位（VEP）及体感诱发电位（SEP）检测，分析检测结果；对15例WD患者进行颅脑MRI检查，并分析检查结果。结果（1）15例WD患者中，12例BAEP异常，临床有明显神经症状患者主要表现为一侧或双侧Ⅲ、V波波峰潜伏期（PL），III-V、I—V波峰间期（IPL）延长，临床无神经症状或症状较轻者，主要表现为一侧或双侧I、Ⅲ波PL，I～Ⅲ波IPL延长；10例VEP异常，主要表现为P100PL延长，其中3例为临床无明显神经症状者；12例SEP异常，主要表现为N20、P40PL、N13．N20IPL延长。（2）15例WD患者颅脑MRI有9例发现异常，病变分布为：壳核9例（60％），苍白球6例（40％），尾状核5例（33．3％），丘脑3例（20％），脑干4例（26．7％）。病灶多表现为长T1长12异常信号，在高磁场MRI中部分病例病灶可在T1、他加权出现高低混杂信号。（3）WD患者脑诱发电位的异常可能发生在临床神经症状及颅脑MRI改变之前。结论脑诱发电位检查对神经系统症状前或轻症患者及WD脑干病变的诊断可能更具价值。脑诱发电位、颅脑MRI可反映脑部损害程度，二者与临床表现三者相结合可能提高WD的早期诊断率。Objective To observe the changes of brain evoked potential and brain MR imaging in 15 patients with Wilson＇s disease（WD）. Methods Fifteen cases of WD and 15 cases of healthy people were included in for brainstem auditory evoked potential （ BAEP）, visual evoked potential （VEP） and somatosensory evoked potential（SEP） examination; 15 cases of WD were included in the study for brain MR imaging examination. Results （1）Abnormal BAEP, VEP and SEP were found unilaterally or bilaterally in these 15 cases of WD. BAEP abnormalities were found in 12 patients,prolonged PL of lII, Vand IPL of III-V, I -V were found in patients with neural symptoms, prolonged PL of I , HI and IPL of I -Ill were found in patients without neural symptoms or with mild neural symptoms： Prolonged latency of P100 was found by VEP in 10 patients, and 3 patients among them without neural symptoms ; prolonged PL of N20, P40 and IPL of N13-N20 were found by SEP in 12 patients. （2）Abnormal signals on brain MRI were found in 9 cases：in putamen,9 cases （ 60 % ）, in globus pallidus, 6 cases （40%）, in caudate nucleus, 5 cases （ 33.3 % ）, in thalamus, 3 cases （20%） ,in brainstem,4 cases（26.7% ）. Most of them had long T1 and long T2 signal intensity,while some had mixed short-long signals. （3） In WD patients, abnormalities of brain evoked potentials could happen before clinical symptoms and brain MRI changes. Conclusion Brain evoked potential is of some value in the early WD diagnosis. Brain evoked potential and brain MRI could reflect the degree of WD brain damage,combination of brain evoked potential, brain MRI and clinical manifestations could increase the early diagnosis rate of WD.

关 键 词：肝豆状核变性 诱发电位 听觉 脑干 诱发电位 视觉 诱发电位 躯体感觉 磁共振成像 

分 类 号：R742.4[医药卫生—神经病学与精神病学] R445.2[医药卫生—临床医学]