Wilson's disease in Taiwan.

Wilson's disease (WD) has been studied in Taiwan since 1960s. The study can be divided into three periods: (1) The first period was 1960s, represented by the work of Dr. JB Tu who worked in the U.S. Naval Medical Research Unit No. 2 (NAMRU-2); (2) The second period was 1970s, represented by the work of Dr. ML Leu who also worked in NAMRU-2. During these two periods, d-penicillamine was introduced to Taiwan via NAMRU-2, primarily as study drug; and (3) The third period was 1980s and afterwards. Tu and Leu reported the clinical manifestations, tissue concentrations of copper, and therapeutic effects of d-penicillamine including cupriuresis, reduction of copper content in tissues, and prognosis. Our studies after 1980s included clinical manifestations, evoked potentials to detect the extent of CNS involvement, effect of superimposed hepatitis B infection on clinical manifestations and prognosis, and WD with cerebral white matter involvement. The present review highlights above investigations.