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JIMD Reports, Volume 25

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JIMD Reports, Volume 25
Springer Berlin Heidelberg

Table of Contents

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    Book Overview
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    Chapter 421 Coenzyme Q10 and Pyridoxal Phosphate Deficiency Is a Common Feature in Mucopolysaccharidosis Type III.
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    Chapter 454 The Pathobiochemistry of Gastrointestinal Symptoms in a Patient with Niemann-Pick Type C Disease
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    Chapter 456 PNPO Deficiency and Cirrhosis: Expanding the Clinical Phenotype?
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    Chapter 457 The Spectrum of Krabbe Disease in Greece: Biochemical and Molecular Findings.
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    Chapter 458 Liver Fibrosis Associated with Iron Accumulation Due to Long-Term Heme-Arginate Treatment in Acute Intermittent Porphyria: A Case Series
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    Chapter 459 Exercise Intolerance and Myoglobinuria Associated with a Novel Maternally Inherited MT-ND1 Mutation.
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    Chapter 461 Vitamin E Improves Clinical Outcome of Patients Affected by Glycogen Storage Disease Type Ib
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    Chapter 462 New Cases of DHTKD1 Mutations in Patients with 2-Ketoadipic Aciduria
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    Chapter 465 Urine Beta2-Microglobulin Is an Early Marker of Renal Involvement in LPI
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    Chapter 466 Improvement of Diffusion Tensor Imaging (DTI) Parameters with Decoppering Treatment in Wilson’s Disease
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    Chapter 467 Screening Mucopolysaccharidosis Type IX in Patients with Juvenile Idiopathic Arthritis
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    Chapter 469 GM2-Gangliosidosis, AB Variant: Clinical, Ophthalmological, MRI, and Molecular Findings
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    Chapter 472 Pitfalls in Diagnosing Neuraminidase Deficiency: Psychosomatics and Normal Sialic Acid Excretion.
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    Chapter 480 Successful Domino Liver Transplantation from a Patient with Methylmalonic Acidemia
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    Chapter 483 Reduction of Plasma Globotriaosylsphingosine Levels After Switching from Agalsidase Alfa to Agalsidase Beta as Enzyme Replacement Therapy for Fabry Disease
Attention for Chapter 466: Improvement of Diffusion Tensor Imaging (DTI) Parameters with Decoppering Treatment in Wilson’s Disease
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Chapter title
Improvement of Diffusion Tensor Imaging (DTI) Parameters with Decoppering Treatment in Wilson’s Disease
Chapter number 466
Book title
JIMD Reports, Volume 25
Published in
JIMD Reports, January 2015
DOI 10.1007/8904_2015_466
Pubmed ID
Book ISBNs
978-3-66-249667-1, 978-3-66-249668-8
Authors

A. Lawrence, J. Saini, S. Sinha, S. Rao, M. Naggappa, P. S. Bindu, A. B. Taly, Lawrence, A., Saini, J., Sinha, S., Rao, S., Naggappa, M., Bindu, P. S., Taly, A. B.

Abstract

This study was undertaken to analyse serially the effects of decoppering therapy on the clinical features, disability and MRI brain including DTI metrics in patients with Wilson's disease. Thirty-five patients with clinically and serologically confirmed neuropsychiatric form of Wilson's disease (WD) on decoppering therapy were followed for a minimum duration of 1 year with serial assessment of their clinical features, disability status and serial MR imaging of the brain including DTI. The cohort included 18 treatment-naïve patients and 17 patients already on decoppering therapy (M/F = 2.18:1). The mean age at which they underwent baseline assessment for this study was 18.6 ± 7.6 years, and follow-up assessment was done after a mean duration of 23.5 ± 8.8 months (range, 12 to 45 months). Along with the overall clinical improvement noted at follow-up, the disability assessed using Chu staging and MSEADL showed significant reduction in the number of patients with severe disability and the mean NSS reducing from 9.74 to 6.37 (p = 0.002). The mean MRI scores showed significantly reduced disease burden from a baseline score of 5.9 (±4.2) to 4.9 (±4.7) in follow-up scans (p < 0.05). Voxel-wise comparison of serial DTI metrics on TBSS (tract-based spatial statistics) analysis showed that the entire cohort had significant (p < 0.05) improvement in all the four parameters (MD, FA, DA and RD) indicated by a decrease in MD, DA and RD values and increase in FA values. Comparison of whole-brain white matter DTI measures between pre- and posttreatment did not show any significant difference (p < 0.05). Patients with Wilson's disease on decoppering therapy showed clinical improvement accompanied with improvement in DTI metrics. Quantitative DTI metrics may be used as surrogate markers of clinical status following initiation of medical therapy in Wilson's disease.

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Geographical breakdown

Country Count As %
Unknown 2 100%

Demographic breakdown

Readers by professional status Count As %
Researcher 1 50%
Other 1 50%
Readers by discipline Count As %
Agricultural and Biological Sciences 1 50%
Engineering 1 50%