Neuropsychological differences were observed between patients with Wilson disease (WD) and healthy controls in India, researchers reported in the Journal of Neurosciences in Rural Practice.
During the small study, the WD patients scored significantly worse on several neuropsychological tests compared to the age- and education-matched controls including the domains of processing speed, frontal executive function, focused attention, verbal memory, and visual memory, in descending order.
Significantly worse scores were observed in the WD cases on the digit span (backward; P =.001), Trail A and B tests (P =.002 and P =.003, respectively), several sections of the auditory verbal learning test (AVLT), and the digit symbol substitution test (DSST; P <.002).
On the AVLT test battery, the WD group performed worse on trials 3-5 (P =.02, P =.04, and P =.044, respectively) as well as on the immediate recall (P = .003) and the delayed recall sections (P <.001). No significant differences were observed in patient scores on digit span (forward) or the first 2 trials of the AVLT.
Read more about WD complications.
When assessing associations between illness parameters and neuropsychological performance, negative correlations between the Chu staging of illness and performance on the DSST along with trials 1 and 2 of the AVLT were observed using a Spearman’s correlation coefficient test. No significant correlation was found between the duration of illness and neuropsychological performance.
“WD, especially when presenting with neurological symptoms, may also have associated neurocognitive dysfunction, especially in frontal executive functions,” the authors said.
“Considering that the manifestation occurs in childhood or early adolescence, the crucial period for the development of one’s scholastic skills, a rapid, bedside, or clinic-based assessment of neurocognitive functions, may be warranted in patients with neurological WD and subsequent cognitive remediation alongside early interventions may lead to improved clinical outcomes and [quality of life],” the authors continued.
A total of 10 patients with WD and 10 matched controls were recruited from a government-run tertiary care hospital in South India. Healthy family members accompanying the patients were used as the matched controls. The median patient age was 17.5 years, compared to 18 for the controls, and 90% of cases were male, compared to 40% of the controls.
Reference
Rangarajan SK, Sugadev SJX, Philip S. Bedside cognitive assessments in Wilson’s disease: comparing cases and matched controls. J Neurosci Rural Pract. 2022;13(4):795-799. doi:10.25259/JNRP-2021-11-25-R2-(2189)
