Sequential MRI changes in Wilson's disease with de-coppering therapy: a study of 50 patients
- 1 September 2007
- journal article
- Published by Oxford University Press (OUP) in The British Journal of Radiology
- Vol. 80 (957), 744-749
- https://doi.org/10.1259/bjr/48911350
Abstract
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.This publication has 15 references indexed in Scilit:
- Wilson's disease: resolution of MRI lesions following long-term oral zinc therapyActa Neurologica Scandinavica, 2009
- Wilson’s disease: cranial MRI observations and clinical correlationNeuroradiology, 2006
- Cranial MR findings in Wilson's diseaseActa Radiologica, 1997
- Cranial MR imaging in Wilson's disease.American Journal of Roentgenology, 1996
- Initial and follow‐up brain MRI findings and correlation with the clinical course in Wilson's diseaseNeurology, 1994
- Wilson's disease: MRI with clinical correlationNeuroradiology, 1994
- Correlation of evoked potential and MRI findings in Wilson's diseaseNeurology, 1993
- Clinical Assessment of 31 Patients With Wilson's DiseaseArchives of Neurology, 1987
- SENSORY EVOKED POTENTIALS IN WILSON'S DISEASEBrain, 1986
- WILSON'S DISEASE AN ANALYSIS OF THE CRANIAL COMPUTERIZED TOMOGRAPHIC APPEARANCES FOUND IN 60 PATIENTS AND THE CHANGES IN RESPONSE TO TREATMENT WITH CHELATING AGENTSBrain, 1981