中国神经再生研究(英文版)

中国神经再生研究(英文版) ›› 2014, Vol. 9 ›› Issue (21): 1912-1916.doi: 10.4103/1673-5374.145360

• 原著:脑损伤修复保护与再生 • 上一篇    下一篇

MRI研究证实肝豆状核变性脑实质发生不可逆性损害：发病后24个月内开始螯合治疗会减轻损害

  

  • 收稿日期:2014-10-12 出版日期:2014-11-15 发布日期:2014-11-15

Reversible lesions in the brain parenchyma in Wilson’s disease confirmed by magnetic resonance imaging: earlier administration of chelating therapy can reduce the damage to the brain

Duško B. Kozić 1, Igor Petrović 2, Marina Svetel 2, Tatjana Pekmezović 3, Aleksandar Ragaji 1, Vladimir S. Kostić 2   

  1. 1 Diagnostic Imaging Center, Institute of Oncology, School of Medicine, University of Novi Sad, Put Doktora Goldmana 4, 21204 Sremska
    Kamenica, Serbia
    2 Institute of Neurology Clinical Centre of Serbia, School of Medicine, University of Belgrade, Dr Suboti?a 6, 11000, Belgrade, Serbia
    3 Institute of Epidemiology, School of Medicine, University of Belgrade, Dr Suboti?a 6, 11000, Belgrade, Serbia
  • Received:2014-10-12 Online:2014-11-15 Published:2014-11-15
  • Contact: Du?ko B. Kozi?, University of Novi Sad, School of Medicine, Institute of Oncology, Diagnostic Imaging Center, Put Doktora Goldmana 4, 21204 Sremska Kamenica, Serbia, dusko.b.kozic@gmail.com.
  • Supported by:

    This work was supported by a grant from the Ministry of Science and Technological Development of Serbia, Scientific Project Number 175090.

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摘要：

我们应用MRI研究螯合治疗肝豆状核变性过程中患者大脑损害恢复情况，并探讨疾病首发后开始治疗的最佳时机。对37例肝豆状核变性患者进行首次MRI检查，其中14例在平均5.7年后进行MRI复查。根据患者进行螯合治疗的时间，将患者分为首次发病后24个月内及≥24个月时开始进行治疗。MRI检查结果显示，发病后24个月内进行治疗的患者脑干和壳核损害恢复情况明显好于发病≥24个月时开始进行治疗者，髓鞘溶解和细胞毒性脑水肿也更为轻微。说明应在肝豆状核变性发病后24个月内进行螯合治疗，可减少大脑实质不可逆性损害的发生。

关键词: 肝豆状核变性, 威尔逊病, 诊断, 螯合治疗, MRI, 延迟诊断, 代谢疾病, 铜代谢障碍, 脑桥鞘髓溶解, 肝硬化

Abstract:

The aim of this study was to evaluate the resolution of brain lesions in patients with Wilson’s disease during the long-term chelating therapy using magnetic resonance imaging and a possible significance of the time latency between the initial symptoms of the disease and the introduction of this therapy. Initial magnetic resonance examination was performed in 37 patients with proven neurological form of Wilson’s disease with cerebellar, parkinsonian and dystonic presentation. Magnetic resonance reexamination was done 5.7 ± 1.3 years later in 14 patients. Patients were divided into: group A, where chelating therapy was initiated < 24 months from the first symptoms and group B, where the therapy started ≥ 24 months after the initial symptoms. Symmetry of the lesions was seen in 100% of patients. There was a significant difference between groups A and B regarding complete resolution of brain stem and putaminal lesions (P = 0.005 and P = 0.024, respectively). If the correct diagnosis and adequate treatment are not established less than 24 months after onset of the symptoms, irreversible lesions in the brain parenchyma could be expected. Signal abnormalities on magnetic resonance imaging might therefore, at least in the early stages, represent reversible myelinolisis or cytotoxic edema associated with copper toxicity.

Key words: nerve regeneration, Wilson’s disease, diagnostic imaging, chelating therapy, magnetic resonance imaging, delayed diagnosis, metabolic disorders, copper toxicity, hepatic encephalopathy, pontine myelinolysis, cirrhosis, neural regeneration