Objective: To present a case of a mild wilson disease with neuropsychiatric features which showed clinical and also imaging deteriotation five months after the chelation treatment with trientine.

Background: Wilson Disease is a rare autosomal recessive inherited disorder of copper metabolism. Once the diagnosis of Wilsons Disease has been established to our patient proper chelation was initiated ALD from AL the available drugs trientine was choosen. Trientine is a chelator agent indicated as an initial therapy with less adverse effects than penicillamine.

Methods: The dosage was initially 300mg daily for the first three months and then 600mg daily in two divided doses for the next two months. as soon as the dosage was increased neurological deterioration has been reported. According the neurological examination enchanced of postural and wing beating tremor had been mentioned as well as gait difficulty with dystonic spasms,mild sensory loss and ataxic speech. The brain MRI was deteriorated in comarison to the previous one obtained before the treatment. A characteristic face of giant panda sign has been described along with lesions in the basal ganglia ,thalamus and midbrain.

Results: The treatment was discontinued but before the urinary copper excretion was evaluated. After two weeks without treatment patient’s clinical condition was improved and urinary copper excertion was reevaluated as well as serum copper both were below of the normal levels.

Conclusions: As a conclusion in this case neurological and brain MRI deteriotation was due to excessive copper removal. So tranditional chelating agents may not be safe when used in mild types of wilson disease and preventive therapy like zinc may be more effective and preferable.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/clinical-and-imaging-deterioration-of-a-mild-case-of-wilsons-disease-after-chelation-therapy/