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  

作　　者：Dusko B.Kozic Igor Petrovic Marina Svetel Tatjana Pekmezovic Aleksandar Ragaji Vladimir S.Kostic 

机构地区：[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 Subotica 6,11000,Belgrade,Serbia [3]Institute of Epidemiology,School of Medicine,University of Belgrade,Dr Subotica 6,11000,Belgrade,Serbia

出　　处：《Neural Regeneration Research》2014年第21期1912-1916,共5页中国神经再生研究（英文版）

基　　金：supported by a grant from the Ministry of Science and Technological Development of Serbia,Scientific Project Number 175090

摘　　要：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 signiifcance 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 ifrst symp-toms 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 signiifcant difference between groups A and B regarding complete resolution of brain stem and putaminal lesions （P= 0.005 andP=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 ex-pected. Signal abnormalities on magnetic resonance imaging might therefore, at least in the early stages, represent reversible myelinolisis or cytotoxic edema associated with copper toxicity.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 signiifcance 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 ifrst symp-toms 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 signiifcant difference between groups A and B regarding complete resolution of brain stem and putaminal lesions （P= 0.005 andP=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 ex-pected. Signal abnormalities on magnetic resonance imaging might therefore, at least in the early stages, represent reversible myelinolisis or cytotoxic edema associated with copper toxicity.

关 键 词：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 

分 类 号：Q983.5[生物学—人类学] O482.531[理学—固体物理]