Neurological Wilson Disease: Clinical Manifestations, Biochemical Profile and Neuro-Radiological Characteristics – A Cohort Study From A Tertiary Care Centre of Bangladesh

Objective: Pediatric Neurological Wilson Disease (WD) is comparatively rare. Very limited study has been done in this disorder particularly from Bangladesh. This study was done to describe the clinical manifestations, biochemical features, neuroimaging and treatment pattern of a cohort of Bangladeshi children with neurological Wilson disease.

Methods: This cross sectional study was conducted in the Department of Pediatric Neurology, Bangabandhu Sheikh Mujib Medical University (BSMMU), from July 2018 to July 2021. Children under 18 years of age having either neurological or combined hepatic and neurological features of WD were included. Clinical manifestations, biochemical features and neuroimaging findings were recorded in preformed data shit.

Results: Total 74 patients were taken as study case. A male predominance was observed. Age of presentation was comparatively late in neuro-hepatic group. Jaundice and cirrhosis of liver was observed in more than two third cases of neuro-hepatic group. Most of the patients of both group presented with deterioration of school performance (100% and 88.89%, respectively), dystonia (95% and 75.63%, respectively), drooling (85% and 75.93%, respectively), altered activity of daily living (85% and 77.78%, respectively), dysphasia (95% and 81.48%, respectively). Patients with neuro-hepatic manifestations had significantly lower serum ceruloplasmin and serum albumin while that of urinary total copper excretion was significantly higher in this group. In MRI of brain, most affected parts were putamen, head of caudate nucleus and thalamus. Conclusion: This study highlights the profile of clinical features, biochemical markers, MRI brain changes and treatment modalities of neurological WD of pediatric population of Bangladesh