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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.

Kozić DB, Petrović I, Svetel M, Pekmezović T, Ragaji A, Kostić VS - Neural Regen Res (2014)

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Diagnostic Imaging Center, Institute of Oncology, School of Medicine, University of Novi Sad, Put Doktora Goldmana 4, 21204 Sremska Kamenica, Serbia.

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.

No MeSH data available.


Related in: MedlinePlus

Effect of 24-month-long treatment delay on irreversible changes in the brain parenchyma in a 37-year-old woman with neurologic form of Wilson's disease.Initial (A–C) and follow-up MR (4.5 years later) images of the brain (D–F) in the patient. 24-month-long treatment delay resulted in marked resolution of the thalamic and brain stem lesions (long arrows) but the presence of irreversible, end-stage degenerative changes in the putamina (short arrows).
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Figure 1: Effect of 24-month-long treatment delay on irreversible changes in the brain parenchyma in a 37-year-old woman with neurologic form of Wilson's disease.Initial (A–C) and follow-up MR (4.5 years later) images of the brain (D–F) in the patient. 24-month-long treatment delay resulted in marked resolution of the thalamic and brain stem lesions (long arrows) but the presence of irreversible, end-stage degenerative changes in the putamina (short arrows).

Mentions: Follow-up MR examination (demographic and clinical characteristics of patients presented in Table 2) revealed complete or partial resolution of the MRI lesions after D-penicillamine treatment in a substantial number of patients with WD in the putamen, caudate nuclei, thalamus, and brainstem (Figure 1). These changes were particularly prominent in mesencephalon and pons and to a lesser degree in basal ganglia. In all three patients with complete resolution, the putaminal lesions were described only on the PDW sequence on initial scanning and they all belong to the group A (patients with introduction of D-penicillamine less than 24 months of the disease onset). In two patients from the group B (patients with delayed onset of such therapy), end-stage neurodegenerative changes were observed in the putaminal periphery (Figure 1A, D). Also, in two patients, focal deposition of paramagnetic substance in putamina and caudate nuclei was found.


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.

Kozić DB, Petrović I, Svetel M, Pekmezović T, Ragaji A, Kostić VS - Neural Regen Res (2014)

Effect of 24-month-long treatment delay on irreversible changes in the brain parenchyma in a 37-year-old woman with neurologic form of Wilson's disease.Initial (A–C) and follow-up MR (4.5 years later) images of the brain (D–F) in the patient. 24-month-long treatment delay resulted in marked resolution of the thalamic and brain stem lesions (long arrows) but the presence of irreversible, end-stage degenerative changes in the putamina (short arrows).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4281431&req=5

Figure 1: Effect of 24-month-long treatment delay on irreversible changes in the brain parenchyma in a 37-year-old woman with neurologic form of Wilson's disease.Initial (A–C) and follow-up MR (4.5 years later) images of the brain (D–F) in the patient. 24-month-long treatment delay resulted in marked resolution of the thalamic and brain stem lesions (long arrows) but the presence of irreversible, end-stage degenerative changes in the putamina (short arrows).
Mentions: Follow-up MR examination (demographic and clinical characteristics of patients presented in Table 2) revealed complete or partial resolution of the MRI lesions after D-penicillamine treatment in a substantial number of patients with WD in the putamen, caudate nuclei, thalamus, and brainstem (Figure 1). These changes were particularly prominent in mesencephalon and pons and to a lesser degree in basal ganglia. In all three patients with complete resolution, the putaminal lesions were described only on the PDW sequence on initial scanning and they all belong to the group A (patients with introduction of D-penicillamine less than 24 months of the disease onset). In two patients from the group B (patients with delayed onset of such therapy), end-stage neurodegenerative changes were observed in the putaminal periphery (Figure 1A, D). Also, in two patients, focal deposition of paramagnetic substance in putamina and caudate nuclei was found.

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Diagnostic Imaging Center, Institute of Oncology, School of Medicine, University of Novi Sad, Put Doktora Goldmana 4, 21204 Sremska Kamenica, Serbia.

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.

No MeSH data available.


Related in: MedlinePlus