The occurrence of neurologic symptoms in patients with purely hepatic Wilson disease (WD) appears to be correlated with insufficient dosing of penicillamine (PCA) and zinc gluconate (ZG) as well as inefficient copper chelating agents, according to a recently published study in BMC Neurology.
Symptomatic patients with WD are classified into neurological, hepatical, and mixed, according to clinical, imaging, and laboratory findings. Liver disease is more common than neurologic involvement. However, if not treated in an appropriate and timely manner, it often develops into a neurologic disease, which is significantly harder to treat.
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In order to contribute to the prevention of the development of neurological symptoms, the authors aimed to study the risk factors involved in the development of neurological symptoms in patients with purely hepatic WD.
The study included 68 patients with purely hepatic WD who received follow-ups for at least a year, during which 14 patients developed neurological symptoms. All of the included patients had complete medical records, including liver function tests, magnetic resonance imaging studies, and doppler ultrasound of the liver and spleen. Patients received treatment with PCA and ZG.
There was no significant difference in patient demographics between both groups, nor hematological values or doppler ultrasound examinations. The authors noted that patients with neurological symptoms had significantly higher AST and ALT levels on average.
Regarding copper metabolism, the authors observed that patients with neurological symptoms had lower adherence to a cooper-free diet and higher urinary cooper. Furthermore, PCA and ZG doses were lower in patients with neurological symptoms.
Statistical analysis with logistic regression analysis showed that insufficient dosing of PCA and ZG influenced the development of neurological symptoms. Although patients with neurological symptoms had lower adherence to diet, statistical analysis did not reveal this to be a risk factor.
The authors hypothesize that the insufficient PCA and ZG doses could have led to increased copper accumulation in the liver, which in turn led to elevated liver enzymes and increased urinary excretion.
“In the anticopper treatment of patients with purely hepatic WD, it is of great significance to promote patients’ educational levels, monitor treatment response, and follow the principle of individualized treatment to prevent the development of neurological symptoms,” the authors concluded.
Reference
Diao, SP, Zhuang, YS., Huang, YQ. et al. Analysis of risk factors for neurological symptoms in patients with purely hepatic Wilson’s disease at diagnosis. BMC Neurol . Published online February 28,2023. doi: 10.1186/s12883-023-03105-w
