Initial Treatment of Patients Who Present with Neurologic and/or Psychiatric Disease

Abstract

From 1956 until the 1990s we essentially had one anticopper drug for Wilson’s disease—penicillamine (64). True, trientine was introduced in 1982 (51), but it was essentially a chelating substitute for penicillamine, to be used only in case of penicillamine toxicity. The medical mentality was very simple—you treated Wilson’s disease with penicillamine. There were no subtleties of using one kind of anticopper drug for one type of patient, and another drug for another type, or varying anticopper drugs for different phases of the disease. You used penicillamine, period. So engrained is that philosophy that fourteen years after we first published (62) on the 25% risk of permanent and severe worsening when penicillamine is used for initial treatment of neurological Wilson’s disease, most physicians still push the penicillamine treatment button when they see a case of Wilson’s disease, no matter the circumstances, neurological presentation or not. The same is true ten years after our first publication (59) on the use of tetrathiomolybdate (TM) as a remarkably effective initial therapy for this type of patient. The same is true four years after the FDA approval of zinc, and zinc is a far safer treatment for the acutely ill neurologic patient than penicillamine. So, Doctors, we now have additional choices of anticopper drugs. It’s time we did a little thinking before we treat a case of Wilson’s disease, and begin customizing our therapy for the patient.