Once
David Goldblatt
Movement disorders can be horrifying. Afflicted persons are solidified or contorted. They may flail so violently that a fork endangers their lives. As a beginning neurologist, I assumed that all such patients curse their fate. Once I got to know Brian, though, I realized that I could be wrong.
Brian and one of his brothers had inherited Wilson’s disease, a rare, genetic movement disorder that had spared their eight siblings.
People who have Wilson’s disease can’t handle dietary copper properly. It accumulates in–and poisons–the kidneys, liver and brain. Avoiding foods rich in copper does not halt the progression of the disease, but it helps. If patients are also treated early and consistently with a drug such as penicillamine, which binds copper and aids in its excretion, they can expect to live a normal lifespan. If not treated, they die young.
Oscar, Brian’s younger brother, was less affected than Brian in his movements and speech. He looked out for Brian in an unusual way: he punched, pushed and made fun of him. (Psychiatric disorders are common in the disease.) Oscar died in his twenties in a car accident. His spleen, swollen because of Wilson’s-related liver disease, ruptured, and he » Continue Reading.
