WILSON’S DISEASE

NEUROLOGICAL MANIFESTATIONS IN PATIENTS PRESENTING TO TERTIARY CARE FACILITY

Abstract

Objectives: To determine the frequency of various neurological manifestations
of Wilson’s disease in patients presenting to tertiary care facility. Study design: Cross sectional
study. Setting: Neurology ward, JPMC, Karachi. Period: 14-03-2012 to 13-09-2012. Patients
and Methods: A total of 150 diagnosed patients of Wilson’s disease between the age 15 and
55 years were recruited. Structured questionnaire was used to collect the data regarding
neurological manifestations of Wilson’s disease along with slit lamp examination for kayser-
Fleischer rings. Data were entered and analyzed in SPSS version 17. Chi-Square test was used
as test of significance. Results: Mean age + S.D of patients was 38.7 + 8.23 years. Most of the
patients (42%) belongs to age between 41 to 50 years. Male to female ratio was 2:1. Tremor
was the most common neurological manifestation (88.7%) followed by dysarthria in 77.3%,
dystonia was present in 59.3%, chorea in 36%, and Parkinsonism in 28.7%. All patients had
the Kayser-Fleischer ring positive. Upon stratification of gender, tremor & dysarthria were more
common in males as compared to females. Only dysarthria was significantly noted to increase
with increasing age up to 5th decade of life and decreased after age of 51 years. (P value
< 0.001). Conclusion: Neurologic manifestations are very common in patients of Wilson’s
disease, tremor being the most common with male predominance.