Bedside cognitive assessments in Wilson’s disease: Comparing cases and matched controls

Abstract

Objectives: Wilson’s disease (WD) is an autosomal recessively inherited disorder with a reported prevalence of 33–68/100,000 in Asian countries not including India. There is a paucity of research in India on prevalence, pattern, and profile of neuropsychological deficits among these patients. The objectives of the study were to profile neuropsychological differences between patients with WD and age- and education-matched healthy controls. Material and Methods: A hospital-based, cross-sectional, and comparative study using strategic combination of neuropsychological tests. Persons with neurological WD receiving IP care over a 3-month period were compared with matched controls. The inclusion criteria were diagnoses of Chu Stage 1 and Chu Stage 2 neurological WD, age 15–45 years, illness of minimum 6 months, and diagnosis confirmed by low serum ceruloplasmin. Exclusion criteria were evidence or clinical suspicion of intellectual disability and past or current psychiatric illness. Results: Median age of patients – 17.5, median age of controls – 18. R software was used to analyze the results. For all cases and controls, time taken to administer the set of tests was always <30 min. Non-parametric tests were chosen considering the data distribution. Statistically significant differences with P < 0.05 are noted in domains of processing speed, frontal executive function, focused attention, verbal, and visual memory in descending order. Conclusion: A strategic compilation of easily performed bedside neuropsychological tests demonstrated differences between the two groups. This combination can be rapidly administered in the clinical setting and hence improve change tracking. This may aid in early identification and hence, earlier initiation of therapy with a possibility of improved clinical outcomes.