Neural Regeneration Research ›› 2014, Vol. 9 ›› Issue (21): 1912-1916.doi: 10.4103/1673-5374.145360

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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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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