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Sequential MRI changes in Wilson's disease with de-coppering therapy: a study of 50 patients

Departments of ¹ Neurology and Neuroimaging and ² Interventional Radiology, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, India

Correspondence: Dr Sanjib Sinha, Associate Professor of Neurology, NIMHANS, Department of Neurology, Hosur Road, Bangalore, Karnataka 5, India. E-mail: sanjib_sinha{at}nimhans.kar.nic.in

Wilson's disease (WD) is clinically and radiologically a dynamic disorder. However, there is a paucity of studies involving sequential MRI changes in this disease with or without therapy

This study looked at serial MRI changes and their clinical correlate in patients with WD

The severity of MRI changes using 1.5 T MRI in 50 patients with WD was graded based on alteration in signal intensity of focal lesions and atrophy. Details of clinical manifestations, Schwab and England Activities of daily living (MSEADL) score, Neurological Symptom Score (NSS) and Chu staging were recorded. Clinical severity and disability scores were correlated with MRI scores using SPSS v10

The mean age at onset of illness and diagnosis was 12.8±5.6 years and 14.4±6.0 years, respectively. At the time of first MRI, patients had been treated for 49.0±77.3 months. At a follow-up of 24.2±12.2 months, clinically 36 patients had improved, 9 remained the same and 5 had worsened. Serial imaging revealed an improvement in MRI parameters in 35 patients, no significant changes in 10, worsening in 4 and an admixture of resolving and evolving changes in 1. The overall MRI score improved from 8.2±5.7 to 5.9±6.6. There was an improvement in measures of disability and impairment in all: Chu stage, 11.5±0.7 to 1.3±0.6; MSEADL score (%), 79.7±27.6 to 88.0±25.4; NSS, 10.6±11.2 to 8.0±11.6, with good clinico-radiological correlation. Patients with extensive changes, white-matter involvement and severe diffuse atrophy had a poor prognosis

In conclusion, the majority of patients with WD showed variable improvement in clinical and MRI features when treated.