TY - JOUR
T1 - Sequential MRI changes in Wilson's disease with de-coppering therapy
T2 - A study of 50 patients
AU - Sinha, Sanjib
AU - Taly, A. B.
AU - Prashanth, L. K.
AU - Ravishankar, S.
AU - Arunodaya, G. R.
AU - Vasudev, M. K.
PY - 2007/9
Y1 - 2007/9
N2 - 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.
AB - 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.
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U2 - 10.1259/bjr/48911350
DO - 10.1259/bjr/48911350
M3 - Article
C2 - 17709362
AN - SCOPUS:35549011999
SN - 0007-1285
VL - 80
SP - 744
EP - 749
JO - British Journal of Radiology
JF - British Journal of Radiology
IS - 957
ER -