Wilson disease: a most unusual patient.

It is generally believed that Wilson disease is an illness of children or young adults. Scheinberg and Sternlieb in their monograph ‘Wilson’s Disease’, based on a large series of patients seen over many years, reported only three patients over the age of 40. In my own series of over 300 patients seen between 1955 and 2000, the oldest patient referred was aged 39 years. However, it is now becoming clear that this disease can present in a much older age group, and, in the past, many patients must have been misdiagnosed. For instance, Peri et al. reported a patient presenting at the age of 60. Ala et al. examined even older patients, one presenting at the age of 70 and the other at 72 years. Walshe et al. in an article on the incidence of malignancies in patients with Wilson disease, noted two patients whose initial signs of Wilson disease occurred at the ages of 56 and 63 years. Ferenci et al. noticed 31 patients with initial neurological signs presenting over the age of 40 and Gow et al. detected 5 of 22 patients over the age of 40. Finally, I have had correspondence from a patient who claimed that her Wilson disease was diagnosed, with liver symptoms, at the age of 80, but it was not possible to get definite confirmation of this diagnosis. However, it is now clear that the age of onset can no longer be considered as eliminating this diagnosis. One patient in my series illustrates how difficult it can be in establishing the correct diagnosis and deciding on what, if any, treatment should be established.