Acute onset neurological symptoms in Wilson disease after traumatic, surgical or emotional events A cross-sectional study
Acute onset neurological symptoms in Wilson disease after traumatic, surgical or emotional events A cross-sectional study
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创伤、手术或情绪事件后威尔逊病急性发作的神经系统症状横断面研究
DOI：
10.1097/md.0000000000015917
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发表时间：
2019-06-01
期刊：
影响因子：
1.6
通讯作者：
Wang, Yu
中科院分区：
文献类型：
--
作者：
Li, Liang-Yong;Zhu, Xiao-Qun;Wang, Yu
由于创伤、手术或情绪事件诱发的Wilson病（WD）急性发作性神经系统症状未见报道，其临床特征也不清楚，本文总结了一种特殊的WD的临床特征，其神经系统症状在创伤、手术、情绪事件后急性发作，或情绪事件。回顾性试点研究。三十-回顾性研究1例以急性神经系统症状为首发症状的WD患者，或在轻度创伤、手术或情绪事件后新出现的肝WD患者。所有患者在常规抗铜治疗后随访半年至1年，神经系统症状表现的平均潜伏期为2.79 ± 1.21小时。最常见的神经系统症状是震颤（74%），所有患者的磁共振成像均检测到基底节（BG）病变。其他区域的病变检出率要低得多。神经症状评分及治疗后恢复与病变部位（BG区和BG+其他脑区）相关。21例患者及时抗铜治疗后神经系统症状改善，6例患者未接受常规抗铜治疗而延迟诊断，当青少年或成人在创伤、手术或情绪刺激后出现急性锥体外系表现时应考虑WD的诊断。及时的抗铜治疗通常会产生良好的预后。
Acute onset neurological symptoms evoked by traumatic, surgical, or emotional events in Wilson disease (WD) have never been reported and its clinical characteristics are unclear.We aimed to summarize the clinical characteristics of a special WD whose neurological symptoms acutely developed after traumatic, surgical, or emotional events.Retrospective pilot study.Thirty-one patients who had acute onset neurological symptom as an initial presentation of WD or a new presentation of hepatic WD after mild trauma, surgery, or emotional events were retrospectively studied. All patients were followed for half to 1 year after regular anti-copper treatment.The averaged latency for neurological symptom presentation was 2.79 +/- 1.21 hours. The most frequent neurological symptoms were tremor (74%) and basal ganglia (BG) lesions were detected on magnetic resonance imaging in all patients. Lesions in other regions were much less frequently detected. Neurological symptom score and its recovery after treatment were correlated with lesion location: BG area and BG plus other brain areas. Neurological symptoms improved in 21 patients who received timely anti-copper treatment but continued to deteriorate in 6 patients who did not accept regular anti-copper treatment for delayed diagnosis.A diagnosis of WD should be considered when adolescents or adults experience acute presentation of extrapyramidal systems after traumatic, surgical, or emotional stimulation. Timely anti-copper therapy usually gives rise to an excellent prognosis.