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01-06-2020 | Wilson's Disease | Original Communication

A study of susceptibility-weighted imaging in patients with Wilson disease during the treatment of metal chelator

Authors: Xiangxue Zhou, Xia Xiao, Xun-hua Li, Hao-ling Qin, Xiao-Yong Pu, Ding-bang Chen, Chao Wu, Li Feng, Xiu-ling Liang

Published in: Journal of Neurology | Issue 6/2020

Abstract

Objective

A randomized-controlled trial comparing study of the changes in brain sensitive-weighted imaging (SWI) of Wilson disease (WD) patients during the treatment with metal chelator was done.

Methods

100 untreated WD patients (80 cases of cerebral type, 20 cases of hepatic type, age 20.13 ± 9.12 years old) and 20 normal controls were selected. Neurological symptoms were scored using the modified Young scale. Liver function tests and copper indices were collected. All study objects received SWI test of the brain. The values of corrected phase (CP) were calculated on SWI. Cerebral-type WD patients were treated with D-penicillamine (DPA) (group 1) or Dimercaptopropane Sulfonate (DMPS) + Dimercaptosuccinic Acid (DMSA) (group 2). Hepatic-type WD patients were treated with DPA (group 3). All patients received annual neurological symptom score, liver function, copper indices, and SWI examination.

Results

At the first year of treatment, score of the modified Young scale in group 2 was lower than that in group 1 (P = 0.023) and lower than that before treatment (P = 0.040). After 2 years of treatment, the score of the modified Young scale in group 1 was lower than that before treatment (P = 0.012). At the second year after treatment, the urinary copper in group 2 was higher than that in group 1 (P = 0.014). Urinary copper was maintained at 200 µg/day in group 1 and 300 µg/day in group 2 after 3 years of treatment. At the first year of treatment, serum copper in group 1 was lower than that in group 2 (P = 0.032). At the first year of treatment, CP values of the pallidum and substantia nigra in group 2 were higher than those in group 1 (P = 0.026, 0.040). At the second year of treatment, CP value of substantia nigra in group 2 was higher than that in group 1 (P = 0.037). After 3 years of treatment, there was no difference in CP values between WD patients and normal controls.

Conclusions

Therapy with DMPS and DMSA improves neurological symptoms of WD patients more quickly and leads to less aggravation, compared with therapy with DPA. The metal content in the brain of WD patients was at a low level after 3 years of treatment. DMPS and DMSA can remove metal from brain tissue faster than DPA.

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Title: A study of susceptibility-weighted imaging in patients with Wilson disease during the treatment of metal chelator
Authors: Xiangxue Zhou
Xia Xiao
Xun-hua Li
Hao-ling Qin
Xiao-Yong Pu
Ding-bang Chen
Chao Wu
Li Feng
Xiu-ling Liang
Publication date: 01-06-2020
Publisher: Springer Berlin Heidelberg
Keywords: Wilson's Disease
Wilson's Disease
Published in: Journal of Neurology / Issue 6/2020
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-020-09746-y

Keynote webinar | Spotlight on medication adherence

Springer Medicine

A study of susceptibility-weighted imaging in patients with Wilson disease during the treatment of metal chelator

Abstract

Objective

Methods

Results

Conclusions

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Objective

Methods

Results

Conclusions

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