[Treatment of chronic Wilson's disease in 2 patients using plasmapheresis--clinico-biochemical observations].

BACKGROUND

The introduction of penicillamine in the treatment of Wilson's disease (hepatolenticular degeneration) was a historical event [1]. D-pericillamine (d-PAM) showed some potential side effects such as myasthenia, kidney toxicity, etc. In previous decade the treatment of Wilson's disease (WD) with zinc sulphate started successfully [4]. Danks [7] described the use of exchanged transfusions, peritoneal dialysis and plasmapheresis in the treatment of young patients with WD in acute liver failure. These patients had acute copper poisoning. The results of this study were beneficial.

METHODS

Therapeutic plasmapheresis (PF) has been used in many diseases in which immunological mechanisms are proved [8, 9]. We started with using PF in the treatment of two young chronic patients with Wilson's disease. The clinical picture of patients became worse, probably due to the decrease in cupriuretic effect of d-PAM. One patient (1) did not take d-PAM regularly. In this study PF was performed with the use of haemonetics V-50 and filter 704. During the treatment with PF, 2000 ml of plasma was always exchanged, i.e. removed. During the treatment with PF the patients were hospitalized at the Department of Neurology and Psychiatry of Children and Young People.

RESULTS

Patient No. 1. A 24-year-old man, born in 1965, came with a coarse tremor. He has been diagnosed as WD at the age of 18. Kayser-Fleischer rings were found in the cornea by slit-lamp examination. The disease began when he was 15 years old with polymorphous difficulties. In time tremor became more severe. It was apparent when the patient made any movement. The first treatment with PF (1) lasted from February 13 to March 16, 1989. The patient had 10 PF without any side effect. During the treatment with PF the patient felt better. After PF-tremor was reduced significantly, and subjective and objective condition ameliorated. The patient (1) was readmitted to the hospital on June 24, 1991 for continuation of the treatment with PF. Anamnestic data and neurological examination revealed progression of the disease. His condition became worse, especially tremor. He could not write a single letter (Figures 1-5) and walk without help of the others. His tremor became the severest when he made any kind of voluntary movement. The second treatment with PF started on June 25, 1991 and finished on July 16, 1991. He received 9 PF in this series. The treatment was without side effects. Tremor was reduced approximately by 15%. Plasma copper examined before and after 9 PF showed different values (Table 2). This difference (2.79 mumol/l; 23.70%) in plasma copper level was removed from circulation. Patient No. 2. A 23-year-old man, born in 1966, came to the hospital with acute exacerbation of WD. At the age of 16 latent psychosis was diagnosed. One year later diagnosis of WD was established in the hospital when he was 17 years old. Kayser-Fleischer rings were found in the cornea by slit-lamp examination. The treatment with d-PAM and other drugs (BAL, symptomatic therapies, sedatives, antidepressants, etc.) has been accompanied with good and long-term remissions and short exacerbation. A few years later exacerbation became longer and longer and worse and worse. He was admitted to the hospital on November 28, 1989 with acute relapse of WD. His condition was very difficult, completely bedridden. The treatment with PF started on December 26, 1989 and lasted to January 25, 1990. The treatment with PF was without d-PAM. He was only given symptomatic therapies. After a few PF he demonstrated side effects with nausea, sometimes vomiting, face sweating, pulse rate of about 120/min while blood pressure was normal. Therefore he was given human albumin in the next day, and no side effects were observed. The removal of little plasma copper from blood circulation correlated well with a small improvement in clinical symtpomatology. The rigidity was reduced and voluntary movements bec