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

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...

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Bibliographic Details
Published in:British journal of radiology 2007-09, Vol.80 (957), p.744-749
Main Authors: SINHA, S, TALY, A. B, PRASHANTH, L. K, RAVISHANKAR, S, ARUNODAYA, G. R, VASUDEV, M. K
Format: Article
Language:English
Subjects:
Adolescent
Adult
Astringents - therapeutic use
Biological and medical sciences
Chelating Agents - therapeutic use
Child
Disease Progression
Female
Health Status Indicators
Hepatolenticular Degeneration - diagnosis
Humans
India
Magnetic Resonance Imaging
Male
Medical sciences
Metabolic diseases
Metals (hemochromatosis...)
Other metabolic disorders
Penicillamine - therapeutic use
Prognosis
Prospective Studies
Zinc Sulfate - therapeutic use
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Summary: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.
ISSN:0007-1285
1748-880X
DOI:10.1259/bjr/48911350