Anwar N¹*, Patel A² and Sheikh S³

¹Assistant Professor, Dept. of Neurology, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh
²Resident, Neurology, Kokilaben Dhirubhai Ambani Hosptal, Mumbai, India
³Clinical Associate, Neurology, Kokilaben Dhirubhai Ambani Hosptal, Mumbai, India

*Corresponding Author: Anwar N, Assistant Professor, Dept. of Neurology, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh.

Received: October 18, 2023; Published: November 23, 2023

Abstract

Background: Wilson’s disease is an autosomal recessive disorder of copper metabolism with various clinical presentations.

Objective: The objective of the study was to asses clinical presentation of Wilson’s disease patients. Method: Total 31 suspected patients of Wilson’s disease attending the Neurology Clinic, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute from August 2022 to June 2023 were included in this study and assessed for clinical presentation.

Results: Out of 31 patients with Wilson’s disease, 20 (64.51%) were males. Almost 83.87% of the patients belonged to younger age group (10-30 years of age). Five (16.12%) presented with hepatic disease only, 07 (22.58%) with neurological disorders only, 01 (3.22%) with neuro-ophthalmological symptoms and 18 patients (58.01%) with mixed features. Out of 25 patients that had neurological disorders, 03 (12%) were found to have dystonia, 03 (12%) presented with ataxia, 02 (8%) had involuntary tremors of hands and feet and 18 presented with mixed neurological features(68%). 19 patients (61.29%) out of 31had K-F ring visible on slit lamp biomicroscopy. In hepatic presentation only 02 had K-F ring (40%), in neurological presentation 04 had K-F ring (57.14%) and in mixed presentation 13 had K-F ring(72.22%).Twenty-four hour urinary copper excretion was positive (level exceeding 100 mg/dl) in 28 cases (90.32%), whereas serum ceruloplasmin level was found lower than 25 mg/dl in 26 patients (83.87%).

Conclusions: Wilson’s disease is more prevalent than previously thought. Neurological manifestation is a fairly common presentation. With appropriate and timely treatment, a significant number of patients improve rapidly.

 Keywords: Wilson’s Disease; Neurological; Presentation

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Citation

Citation: Anwar N., et al. “Clinical Presentation of Wilson’s Disease Patients Attending a Tertiary Level Hospital”. Acta Scientific Neurology 6.12 (2023): 14-17.

Copyright

Copyright: © 2023 Anwar N., et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.