多模态诱发电位在确定威尔逊病亚临床神经受累中的诊断价值。

The diagnostic value of multimodal evoked potentials in the determination of subclinical neurological involvement of Wilson's disease.

机构信息

Department of Pediatric Gastroenterology, Hepatology and Nutrition, Ege University, Izmir, Turkey.

出版信息

Eur J Gastroenterol Hepatol. 2012 Jun;24(6):627-32. doi: 10.1097/MEG.0b013e3283526f81.

DOI:10.1097/MEG.0b013e3283526f81

PMID:22433793

Abstract

AIMS

Wilson's disease (WD) is a progressive degeneration of hepatolenticular tissue caused by excessive tissue-damaging copper accumulation and in which liver involvement most frequently presents in childhood. Neurological signs also accompany liver disease with time. However, subclinical neurological involvement may occur earlier and diagnostic methods that reveal this subclinical involvement are not well established. The aim of the current study is to assess the subclinical neurological involvement by using multimodality evoked potential (EP) measurements and to explore the relationship between neurological disease and the severity of liver damage.

METHODS

The patient group included 28 children (mean age 11.8 ± 2.9 years, range 5.5-17) diagnosed with WD and a control group included 24 age-matched healthy children. Multimodality EP tests (Nihon Kohden Neuropack 8 4200K) of both groups were performed at the Department of Neurology Electrophysiology Laboratory of Ege University.

RESULTS

At least one abnormal EP value was observed in 53.5% of the children in the patient group. At least on one side, there were abnormal values for visual evoked potential (VEP) P100, brainstem auditory evoked potential (BAEP) and somatosensory evoked potential (SEP), where the ratios were 25, 28.5, and 11%, respectively. Absolute latency values of patients with right-side VEP P100 and left-side BAEP I, and the interpeak latency values of right-side BAEP I-III were significantly high. The difference in right-side BAEP I-III interpeak latency between cirrhotic and noncirrhotic groups was found to be statistically significant (P<0.05).

CONCLUSION

The EP examinations can be an indicator of subclinical brain damage in non-neurological WD; however, cirrhosis because of WD does not cause an increase in the EP values. Detection of changes in the EP values periodically, especially at the time of diagnosis and during the treatment follow-up, may be valuable for revealing subclinical impairment.

摘要

目的

威尔逊病（WD）是一种由组织损伤性铜蓄积引起的进行性肝豆状核变性，肝受累在儿童期最常发生。随着时间的推移，神经病变也会伴随肝脏疾病出现。然而，亚临床神经受累可能更早发生，并且尚未建立揭示这种亚临床受累的诊断方法。本研究的目的是通过使用多模态诱发电位（EP）测量来评估亚临床神经受累，并探讨神经疾病与肝损伤严重程度之间的关系。

方法

患者组包括 28 名（平均年龄 11.8 ± 2.9 岁，范围 5.5-17）被诊断为 WD 的儿童，对照组包括 24 名年龄匹配的健康儿童。两组均在伊兹密尔大学神经科电生理实验室进行多模态 EP 测试（尼高顿神经包 84200K）。

结果

患者组中有 53.5%的儿童至少有一项 EP 值异常。至少有一侧视觉诱发电位（VEP）P100、脑干听觉诱发电位（BAEP）和体感诱发电位（SEP）存在异常值，分别为 25%、28.5%和 11%。右侧 VEP P100 和左侧 BAEP I 的绝对潜伏期值以及右侧 BAEP I-III 的峰间潜伏期值较高。肝硬化和非肝硬化组之间右侧 BAEP I-III 峰间潜伏期值的差异具有统计学意义（P<0.05）。