Plasma Neurofilament Light as a Biomarker of Neurological Involvement in Wilson's Disease.

ABSTRACT:

Background

Outcomes are unpredictable for neurological presentations of Wilson's disease (WD). Dosing regimens for chelation therapy vary and monitoring depends on copper indices, which do not reflect end-organ damage.

Objective

To identify a biomarker for neurological involvement in WD.

Methods

Neuronal and glial-specific proteins were measured in plasma samples from 40 patients and 38 age-matched controls. Patients were divided into neurological or hepatic presentations and those with recent neurological presentations or deterioration associated with non-adherence were subcategorized as having active neurological disease. Unified WD Rating Scale scores and copper indices were recorded.

Results

Unlike copper indices, neurofilament light (NfL) concentrations we

SUBMITTER: Shribman S 

PROVIDER: S-EPMC8436757 | biostudies-literature | 2021 Feb

REPOSITORIES: biostudies-literature