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Exchangeable copper: a reflection of the neurological severity in Wilson's disease.

Abstract：

BACKGROUND AND PURPOSE:The severity of Wilson's disease (WD) is linked to free copper accumulating in the liver and brain. Exchangeable copper (CuEXC) is a new technique to determine plasmatic copper and is useful in the diagnosis of WD. It is hypothesized that it may also enable a good evaluation of extra-hepatic involvement and its severity. METHODS:Forty-eight newly diagnosed WD patients were prospectively evaluated using hepatic, neurological, ophthalmological and brain magnetic resonance imaging (MRI) scores. Three phenotypic presentations were distinguished: pre-symptomatic, hepatic and extra-hepatic. CuEXC was determined in addition to standard copper assays before decoppering therapy. Correlations between biological parameters and the different scores were determined and compared in the hepatic and extra-hepatic groups. RESULTS:Extra-hepatic patients had significantly higher CuEXC values than those with the hepatic form (P < 0.0001). The overall ability of CuEXC to separate the two forms was satisfactory, with an area under the curve of 0.883 (95% confidence interval 0.771-0.996) and an optimal threshold for extra-hepatic diagnosis of 2.08 μmol/l (sensitivity 85.7%; specificity 94.1%). In extra-hepatic patients, CuEXC was the only biological marker to be positively correlated with the Unified Wilson Disease Rating Score (r = 0.45, P = 0.016), the Kayser-Fleischer ring score (r = 0.46, P = 0.014) and the brain MRI score (r = 0.38, P = 0.048), but it was not correlated with the hepatic score. CONCLUSIONS:Exchangeable copper determination is useful when diagnosing WD as a value >2.08 μmol/l is indicative of the severity of the extra-hepatic involvement. In the case of purely hepatic presentation, atypical or mild neurological signs, it should encourage physicians to search for lesions in the brain and eyes.

journal_name

Eur J Neurol

journal_title

European journal of neurology

authors

Poujois A,Trocello JM,Djebrani-Oussedik N,Poupon J,Collet C,Girardot-Tinant N,Sobesky R,Habès D,Debray D,Vanlemmens C,Fluchère F,Ory-Magne F,Labreuche J,Preda C,Woimant F

doi

10.1111/ene.13171

subject

Has Abstract

pub_date

2017-01-01 00:00:00

pages

154-160

issue

eissn

1351-5101

issn

1468-1331

journal_volume

pub_type

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Exchangeable copper: a reflection of the neurological severity in Wilson's disease.