| Popis: |
Wilson's disease (WD) is a complex metabolic disorder caused by disruptions to copper regulation within the body, leading to an unregulated accumulation of copper within various tissues. A less understood organ affected by the collection of copper is the brain, which further leads to the generation of oxygen free radicals and resultant demyelination. Healthcare providers need to keep the neurological form of WD in their list of differentials when patients present with diverse neurological manifestations. Being able to distinguish the characteristic disease presentation with a thorough history and physical and neurological examination will be the initial step to diagnosis. A high clinical disease suspicion of WD should warrant further investigation by laboratory workup and imaging modalities to support the clinical findings and confirm the diagnosis of WD. Once a WD diagnosis is established, the healthcare provider should treat for the underlying biological process of WD and symptomatically. This review article discusses the epidemiology and pathogenesis of the neurological form of Wilson's disease, its clinical and behavioral implications, diagnostic features, and treatment modalities, further aiding healthcare professionals in an early diagnosis and management strategies. |
