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

Sanjib Sinha, A. B. Taly, L. K. Prashanth, S. Ravishankar, G. R. Arunodaya, M. K. Vasudev

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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.

Original languageEnglish
Pages (from-to)744-749
Number of pages6
JournalBritish Journal of Radiology
Volume80
Issue number957
DOIs
Publication statusPublished - Sep 2007

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Hepatolenticular Degeneration
Activities of Daily Living
England
Therapeutics
Atrophy
Age of Onset

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Medicine(all)

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Sequential MRI changes in Wilson's disease with de-coppering therapy : A study of 50 patients. / Sinha, Sanjib; Taly, A. B.; Prashanth, L. K.; Ravishankar, S.; Arunodaya, G. R.; Vasudev, M. K.

In: British Journal of Radiology, Vol. 80, No. 957, 09.2007, p. 744-749.

Research output: Contribution to journalArticle

Sinha, Sanjib ; Taly, A. B. ; Prashanth, L. K. ; Ravishankar, S. ; Arunodaya, G. R. ; Vasudev, M. K. / Sequential MRI changes in Wilson's disease with de-coppering therapy : A study of 50 patients. In: British Journal of Radiology. 2007 ; Vol. 80, No. 957. pp. 744-749.
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