Department of Neurology, University Hospital "Alexandrovska," Medical University Sofia, Sofia, Bulgaria.
Department of Neurology, University Hospital Zurich, Zurich, Switzerland.
J Clin Neurophysiol. 2022 Sep 1;39(6):510-512. doi: 10.1097/WNP.0000000000000797. Epub 2020 Nov 10.
Wilson disease (WD) is an inherited disorder of copper metabolism presenting with a variety of symptoms but commonly as a liver or neuropsychiatric disease. Abnormal evoked responses are constantly found among patients with neurologic manifestations and sometimes in patients with hepatic presentation or in presymptomatic siblings. The aim of our study was to assess visual and brainstem auditory evoked potentials (BAEP) in patients with various presentation of WD.
Visual evoked potentials (VEP) were performed in 36 WD patients and BAEP were done in 37 WD patients.
Brainstem auditory evoked potentials were normal in patients with isolated hepatic presentation, whereas they were abnormal in 93.5% of patients with neurologic symptoms. There was significant prolongation of the latencies of the III and V waves and of the interpeak III-V and I-V latencies in comparison with the healthy controls (T-test P = 0). Abnormal VEP were observed in 81% of the patients including six of seven neurologically asymptomatic patients. The values of N75, P100, and N145 latencies were significantly longer in all patients than in healthy controls (T-test).
The data showed that VEP and BAEP are more frequently abnormal in WD than previously reported. The abnormal VEP and BAEP even without clinical signs and brain MRI abnormalities point to subclinical involvement of visual and auditory pathways caused by copper toxicity. Because VEP and BAEP are noninvasive and widely available, they should be performed in all patents with WD.
威尔逊病（WD）是一种遗传性铜代谢紊乱疾病，表现出多种症状，但通常表现为肝脏或神经精神疾病。有神经表现的患者和有时有肝脏表现或无症状的兄弟姐妹中经常发现异常的诱发电位。我们的研究目的是评估各种 WD 表现患者的视觉和脑干听觉诱发电位（BAEP）。
对 36 例 WD 患者进行视觉诱发电位（VEP）检查，对 37 例 WD 患者进行 BAEP 检查。
孤立性肝表现患者的脑干听觉诱发电位正常，而有神经症状的患者中 93.5%异常。与健康对照组相比，III 波和 V 波的潜伏期以及 III-V 和 I-V 潜伏期的峰间潜伏期显著延长（T 检验 P=0）。异常 VEP 见于 81%的患者，包括 7 例无症状神经患者中的 6 例。与健康对照组相比，所有患者的 N75、P100 和 N145 潜伏期均显著延长（T 检验）。
数据表明，WD 患者的 VEP 和 BAEP 比以前报道的更常异常。异常的 VEP 和 BAEP 即使没有临床症状和脑 MRI 异常，也表明铜毒性引起的视觉和听觉通路的亚临床受累。由于 VEP 和 BAEP 是非侵入性的，且广泛可用，因此应在所有 WD 患者中进行。
