Aspects of cuprogenic disorder in Wilson's Disease in India

A brief account is given of some aspects of Wilson's disease in India, studied during the periods 1959 to 1967 and 1970 to 1978, with emphasis on disordered parameters of copper metabolism. Among the latter, serum copper oxidase (representing caeruloplasmin) was found to be the most constant laboratory evidence of Wilson's disease. It wad drastically lowered in all patients in both periods (25 and 44 patients respectively), when compared to its level in the parents and siblings of the patients, in other neurological patients and in normal subjects serving as controls. The other constantly involved parameter was direct-reacting serum copper which was highly significantly elevated in the patients compared to the other groups. Most of the patients presented with a predominantly neurological form of the disease, smaller groups presenting with predominantly osseomuscular or hepatic forms. The mean age of patients in both periods was approximately 13 years.