Hepatolenticular Degeneration; Wilson
Conditions
Keywords
DWI-ADC matching, Wilson disease, clinical value, short-term prognosis
Brief summary
In earlier studies, it was found that patients of Wilson disease with new diagnosed who only has neurological symptoms often had DWI hyper-intensity in brain MRI, which was more common in putamen and midbrain, indicating that the disease was in the acute stage. However, many patients had ADC hyper-intensity or hypo-intensity at the same time, and the two different signals represented different disease processes from an imaging perspective. The former indicating T2 penetration effect, and the latter represents diffusion limitation, which indicating the presence of local inflammation, edema, etc. Whether the signal changes of these two different matching modes have guiding significance for the early de-copper treatment for WD, one is the core point of our study. We hope to exploring the predictive value about DWI-ACD signal matching for symptoms changes in the earlier time of de-copper treatment through this study.
Interventions
None listed
Sponsors
Study design
Eligibility
Inclusion criteria
1. Patients had been previously diagnosed with WD who both had DWI hyper-intensity and ADC hyper-intensity or hypo-intensity at the same time; 2. Patients who accept de-copper treatment in hospitalization for 4 weeks or more.
Exclusion criteria
1. Patients who only with liver injury; 2. Severe complications; 3. Combined with other diseases.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| UWDRS scales | Before and 4 weeks after treatment | It is used to evaluate the changes of patients' nervous system symptoms |
| 24h urine copper | Before and 4 weeks after treatment | used to evaluate the changes of patients' nervous system symptoms |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Semi-quantitative brain MRI score | Before de-copper treatment | It is used to evaluate the changes of patients' nervous system symptoms |
| ALT, AST and other liver function test | Before and 4 weeks after treatment | used to evaluate the changes of patients' nervous system symptoms |
Countries
China