Abstrakt
Introduction: Wilson's disease (WD) is a neurodegenerative disorder that presents mainly with liver symptoms, but may be accompanied by various neurological manifestations. WD can be successfully treated with chelators or zinc salts to correct the abnormal copper metabolism, which usually improves both liver function and the neurological deficits; however, improvement in neurological deficits can take years.
Patients may initially present with severe neurological deficits that affect activities of daily living. Consequently, therapy to alleviate the neurological symptoms is needed.
Treatment choices are often based on experience with other disorders with symptoms clinically similar to WD. Areas covered: Apart from general recommendations on the anti-copper therapy and liver transplantation in patients with WD, no guidelines for managing the neurological symptoms exist.
Based on mainly case reports, this review describes the most frequent neurological symptoms and their possible treatments. Expert opinion: There is no strong evidence regarding treatment of neurological disturbances in patients with WD.
Several case studies indicate that drugs used to treat movement disorders such as tremor, parkinsonism, or dystonia may also alleviate these symptoms in patients with WD. In the case of severe, disabling, neurological symptoms, the treatment options for symptoms should always be discussed with the patient.