MRI研究证实肝豆状核变性脑实质可逆性损害：早期开始螯合治疗会减轻损害

MRI研究证实肝豆状核变性脑实质可逆性损害：早期开始螯合治疗会减轻损害
肝豆状核变性又称威尔逊病，是一种常染色体隐性遗传的铜代谢障碍疾病，由于铜在体内过度蓄积，损害肝、脑等器官而致病。

临床主要表现之一即为神经系统症状。

T2加权成像显示基底节、脑干或丘脑信号强度减低。

早期确诊治疗本病，可使预后大为改观。

那么发病多长时间内进行治疗可更有效减轻大脑实质不可逆性损害呢？为解答这一问题。

塞尔维亚Novi Sad大学医学院Duško B. Kozić所在团队应用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。

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，可减少大脑实质可逆性损害的发生。

相关文章发表于《中国神经再生研究（英文版）》杂志2014年11月第21期。

Article: "Reversible lesions in the brain pare nchyma in Wilson’s disease confirmed by magnetic resonance imaging: earlier administration of chelating therapy can reduce the damage to the brain" by Duško B. Kozić1, Igor Petrović2, Marina Svetel2, Tatjana Pekmezović3, Aleksandar Ragaji1, Vladimir S. Kos tić2 (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 Subotića 6, 11000, Belgrade, Serbia; 3 Institute of Epidemiology, School of Medicine, University of Belgrade, Dr Subotića 6, 11000, Belgrade, Serbia)
Kozić DB, Petrović I, Svetel M, Pekmezović T, Ragaji A, Kostić VS. Reversible lesions in the brain paren chyma in Wilson’s disease confirmed by magnetic resonance imaging: earlier administration of chelating therapy can reduce the damage to the brain. Neural Regen Res. 2014;9(21):1912-1916.
欲获更多资讯：Neural Regen Res
Early chelating therapy reduces reversible lesions to the brain in WD as confirmed by MRI
Wilson's disease (WD) is a rare inherited disorder that causes too much copper to accumulate in your liver, brain and other vital organs. It manifests as neurological symptoms. T2-weighted images show decreased signal intensity in the basal ganglia, brain stem or thalamus. Early diagnosis and treatment can greatly improve the prognosis. At what time after disease onset does the treatment can greatly reduce reversible lesions in the brain parenchyma in Wilson’s disease? To solve this problem, Duško B. Kozid, Institute of Oncology, School of Medicine, University of Novi Sad, Serbia and his colleagues observed the recovery from cerebral injuries in patients with WD after administration of chelating therapy using MRI examination and simultaneously investigated the optimal treatment time after treatment. Initial MRI examination was performed in 37 patients and MRI was performed again in 14 of them after an average of 5.7 years. The recovery of lesions in the brain stem and putaminal lesions was superior to, the myelinolisis or cytotoxic edema was milder, in patients who received chelating therapy starting < 24 months from the first symptoms and in those who received chelating therapy starting ≥ 24 months after the initial symptoms. Chelating therapy should be started less than 24 months after onset of the symptoms, which can greatly reduce the reversible lesions in the brain parenchyma. These results were published in Neural Regeneration Research (Vol. 9, No. 21, 2014).
Article: "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" by Duško B. Kozić1, Igor Petrović2, Marina Svetel2, Tatjana Pekmezović3, Aleksandar Ragaji1, Vladimir S. Kostić2 (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 Subotića 6, 11000, Belgrade, Serbia; 3 Institute of Epidemiology, School of Medicine, University of Belgrade, Dr Subotića 6, 11000, Belgrade, Serbia)
Kozić DB, Petrović I, Svetel M, Pekmezović T, Ragaji A, Kostić VS. 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. Neural Regen Res. 2014;9(21):1912-1916.。