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Treatment of Wilson Disease With Ammonium Tetrathiomolybdate: IV. Comparison of Tetrathiomolybdate and Trientine in a Double-Blind Study of Treatment of the Neurologic Presentation of Wilson Disease

Treatment of Wilson Disease With Ammonium Tetrathiomolybdate: IV. Comparison of Tetrathiomolybdate and Trientine in a Double-Blind Study of Treatment of the Neurologic Presentation of Wilson Disease

George J Brewer, Fred Askari, Matthew T Lorincz, Martha Carlson, Michael Schilsky, Karen J Kluin, Peter Hedera, Paolo Moretti, John K Fink, Roberta Tankanow, Robert B Dick, Julia Sitterly

Arch Neurol. 2006 Apr;63(4):521-7.

PMID: 16606763

Abstract:

Objective:
To compare tetrathiomolybdate and trientine in treating patients with the neurologic presentation of Wilson disease for the frequency of neurologic worsening, adverse effects, and degree of neurologic recovery.
Design:
A randomized, double-blind, controlled, 2-arm study of 48 patients with the neurologic presentation of Wilson disease. Patients either received 500 mg of trientine hydrochloride 2 times per day or 20 mg of tetrathiomolybdate 3 times per day with meals and 20 mg 3 times per day between meals for 8 weeks. All patients received 50 mg of zinc 2 times per day. Patients were hospitalized for 8 weeks, with neurologic and speech function assessed weekly; discharged taking 50 mg of zinc 3 times per day, and returned annually for follow-up.
Setting:
A university hospital referral setting.
Patients:
Primarily newly diagnosed patients with Wilson disease presenting with neurologic symptoms who had not been treated longer than 4 weeks with an anticopper drug.
Intervention:
Treatment with either trientine plus zinc or tetrathiomolybdate plus zinc.
Main outcome measures:
Neurologic function was assessed by semiquantitative neurologic and speech examinations. Drug adverse events were evaluated by blood cell counts and biochemical measures.
Results:
Six of 23 patients in the trientine arm and 1 of 25 patients in the tetrathiomolybdate arm underwent neurologic deterioration (P<.05). Three patients receiving tetrathiomolybdate had adverse effects of anemia and/or leukopenia, and 4 had further transaminase elevations. One patient receiving trientine had an adverse effect of anemia. Four patients receiving trientine died during follow-up, 3 having shown initial neurologic deterioration. Neurologic and speech recovery during a 3-year follow-up period were quite good.
Conclusion:
Tetrathiomolybdate is a better choice than trientine for preserving neurologic function in patients who present with neurologic disease.

Chemicals Related in the Paper:

Catalog Number	Product Name	Structure	CAS Number	Price
AP38260014-A	Trientine hydrochloride		38260-01-4	Price

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