eMedicine Specialties > Neurology > Movement and Neurodegenerative Diseases

Wilson Disease: Follow-up

Author: Celia H Chang, MD, Associate Health Sciences Clinical Professor, Department of Neurology, University of California at Davis
Contributor Information and Disclosures

Updated: Nov 26, 2008

Follow-up

Further Inpatient Care

Treatment of fulminant liver disease

  • Plasma exchange and exchange transfusion as well as peritoneal dialysis with albumin 1-1.5 g/dL and penicillamine 200 mg/dL.
  • Intravenous penicillamine or trientine can also be given.
  • Almost all patients need liver transplantation (see Staging).

Prognosis

  • Improvement usually begins 5-6 months after the start of therapy and continues for about 24 months. The deficits present at 24 months are likely to be permanent.
  • Psychiatric symptoms can resolve, and many neurologic symptoms can improve or resolve as well. However, patients usually have cirrhosis even if liver function is normal. Patients can have complications related to cirrhosis, such as portal hypertension, varices, and hypersplenism resulting in leukopenia and thrombocytopenia.
  • Merle et al20 found that patients presenting with neuropsychiatric symptoms were more likely to have a longer delay to definitive diagnosis (44.4 mo vs 14.4 mo), progression of symptoms despite treatment, and a poorer outcome than patients presenting with hepatic symptoms. Treatment side effects also occurred in 74.4% of their 163 patients. Patients also presented at a younger age with hepatic symptoms (15.5 y vs 20.2 y).
  • Patients who present with fulminant liver failure have a mortality rate as high as 70%.

Patient Education

For excellent patient education resources, visit eMedicine's Liver, Gallbladder, and Pancreas Center and Hepatitis Center. Also, see eMedicine's patient education article, Cirrhosis.

Miscellaneous

Special Concerns

  • Noncompliance is an important problem.
  • In 1 study, occasional noncompliance was reported in 25% of patients, and serious problems with regular noncompliance were found in 10% of patients though patients received free medication and care and though urinary copper values were monitored every 6 months.
  • Sinha and Taly21 reported that 44 of 45 patients on zinc and penicillamine maintenance therapy remained stable after penicillamine was discontinued but 1 patient worsened. Mishra et al22 reported 2 cases of disease progression from presymptomatic to symptomatic disease and biochemical abnormalities despite treatment with zinc. The 2 children both responded to penicillamine.
  • Narayan and Kaveer23 reported a 13-year-old child with hypodensity of the white matter of the frontal, parietal, and temporal lobes consistent with demyelination and both generalized atrophy of the cerebral hemispheres and atrophy of the basal ganglia after 4 years of treatment with 750 mg/d penicillamine, which is equivalent to the adult maintenance dose, and 280 mg/d of zinc, which is nearly double the adult maintenance dose. 

    The authors noted that the serum ceruloplasmin was virtually undetectable and the serum copper (16 μg/dL) was much lower than the normal range of 80-120 μg/dL. They thought the CT changes were due to hypocupremia. The child presented at age 9 years with difficulty using hands and ambulating and with slurred speech for 6 months. He did have basal ganglia atrophy on the initial head CT. On follow-up examination, the KF ring was still present on the superior and inferior margins of both corneae and other abnormalities were apparent.
  • Walshe24 reported that in 67 patients seen from 1948-2000, diagnostic failure was the principal cause of death. Multiple other causes of death included poor compliance and the development of malignant disease after 10 years of follow-up.
 


More on Wilson Disease

Overview: Wilson Disease
Differential Diagnoses & Workup: Wilson Disease
Treatment & Medication: Wilson Disease
Follow-up: Wilson Disease
References
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References

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Further Reading

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Keywords

Wilson's disease, hepatolenticular degeneration, liver cirrhosis, copper overload, Kayser-Fleischer rings, KF rings, Bedlington terrier, disease of copper metabolism, Long Evans Cinnamon rat, LEC rat, toxic milk mouse, MNK, WND

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Contributor Information and Disclosures

Author

Celia H Chang, MD, Associate Health Sciences Clinical Professor, Department of Neurology, University of California at Davis
Celia H Chang, MD is a member of the following medical societies: American Academy of Neurology and Child Neurology Society
Disclosure: Nothing to disclose.

Medical Editor

Christopher Luzzio, MD, Clinical Assistant Professor, Department of Neurology, University of Wisconsin at Madison
Christopher Luzzio, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Nestor Galvez-Jimenez, MD, MSc, MHA, Chairman, Department of Neurology, Program Director, Movement Disorders, Department of Neurology, Division of Medicine, Cleveland Clinic Florida
Nestor Galvez-Jimenez, MD, MSc, MHA is a member of the following medical societies: American Academy of Neurology, American College of Physicians, and Movement Disorders Society
Disclosure: Nothing to disclose.

CME Editor

Matthew J Baker, MD, Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital
Matthew J Baker, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Chief Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

 
 
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