Wilson's disease: two treatment modalities. Correlations to pretreatment and posttreatment brain MRI.
BACKGROUND
Brain magnetic resonance imaging (MRI) studies on Wilson's disease (WD) show lack of correlations between neurological and neuroimaging features. Long-term follow-up reports with sequential brain MRI in patients with neurological WD comparing different modalities of treatment are scarce.


METHODS
Eighteen patients with neurological WD underwent pretreatment and posttreatment brain MRI scans to evaluate the range of abnormalities and the evolution along these different periods. All patients underwent at least two MRI scans at different intervals, up to 11 years after the beginning of treatment. MRI findings were correlated with clinical picture, clinical severity, duration of neurological symptoms, and treatment with two different drugs. Patients were divided into two groups according to treatment: D: -penicillamine (D-P), zinc (Zn), and Zn after the onset of severe intolerance to D-P.


RESULTS
MRI scans before treatment showed, in all patients, hypersignal intensity lesions on T2- and proton-density-weighted images bilaterally and symmetrically at basal nuclei, thalamus, brain stem, cerebellum, brain cortex, and brain white matter. The most common neurological symptoms were: dysarthria, parkinsonism, dystonia, tremor, psychiatric disturbances, dysphagia, risus sardonicus, ataxia, chorea, and athetosis.


CONCLUSIONS
From the neurological point of view, there was no difference on the evolution between the group treated exclusively with D-P and the one treated with Zn. Analysis of MRI scans with longer intervals after the beginning of treatment depicted a trend for neuroimaging worsening, without neurological correspondence, among patients treated with Zn. Neuroimaging pattern of evolution was more favorable for the group that received exclusively D-P.
da Costa
Maria do Desterro Leiros
Mdo D
Movement Disorders Unit, Federal University of Paraiba, Paraíba, Brazil. mleiros@uol.com.br
Spitz
Mariana
M
Bacheschi
Luiz Alberto
LA
Leite
Claudia Costa
CC
Lucato
Leandro Tavares
LT
Barbosa
Egberto Reis
ER
D000293:Adolescent; D000328:Adult; D001921:Brain / Q000187:drug effects / Q000473:pathology*; D002491:Central Nervous System Agents / Q000494:pharmacology*; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D006527:Hepatolenticular Degeneration / Q000188:drug therapy* / Q000473:pathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D010396:Penicillamine / Q000494:pharmacology*; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult; D017967:Zinc Compounds / Q000494:pharmacology*
D002491:Central Nervous System Agents; D017967:Zinc Compounds; D010396:Penicillamine