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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.
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| Measure | Description | Time Frame |
|---|---|---|
| UWDRS scales | It is used to evaluate the changes of patients' nervous system symptoms | Before and 4 weeks after treatment |
| 24h urine copper | used to evaluate the changes of patients' nervous system symptoms | Before and 4 weeks after treatment |
| Measure | Description | Time Frame |
|---|---|---|
| Semi-quantitative brain MRI score | It is used to evaluate the changes of patients' nervous system symptoms | Before de-copper treatment |
| ALT, AST and other liver function test | used to evaluate the changes of patients' nervous system symptoms |
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Inclusion Criteria:
Patients had been previously diagnosed with WD who both had DWI hyper-intensity and ADC hyper-intensity or hypo-intensity at the same timeï¼›
Patients who accept de-copper treatment in hospitalization for 4 weeks or more.
Exclusion Criteria:
Combined with other diseases.
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WD should be considered in patients with unexplained liver disease, neurological symptoms (especially extrapyramidal symptoms), or psychiatric symptoms. Age of onset cannot be used as a basis for diagnosing or ruling out WD.
Among these, patients had been previously diagnosed with WD who both had DWI hyper-intensity and ADC hyper-intensity or hypo-intensity at the same time. At the time of admission, the Unified Wilson Disease Rating Scale (UWDRS) and cranial MRI examinations were conducted to assess the state of the disease. All patients received Cu-chelating therapy according to the Chinese guidelines for the diagnosis and treatment of WD (Neurogenetics Group and Neurology Branch of Chinese Medical Association, 2021). During the course of treatment, serum copper, ceruloplasmin, and urine copper at 24 h before treatment and the highest urine copper at 24 h during treatment were tested to determine the efficacy of treatment.
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| The affiliated hospital of Institute of Neurology in Anhui University of Chinese Medicine | Hefei | Anhui | 230001 | China |
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| ID | Term |
|---|---|
| D006527 | Hepatolenticular Degeneration |
| ID | Term |
|---|---|
| D008107 | Liver Diseases |
| D004066 | Digestive System Diseases |
| D001480 | Basal Ganglia Diseases |
| D001927 | Brain Diseases |
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| Before and 4 weeks after treatment |
| D002493 |
| Central Nervous System Diseases |
| D009422 | Nervous System Diseases |
| D020739 | Brain Diseases, Metabolic, Inborn |
| D001928 | Brain Diseases, Metabolic |
| D009069 | Movement Disorders |
| D020271 | Heredodegenerative Disorders, Nervous System |
| D019636 | Neurodegenerative Diseases |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D008661 | Metabolism, Inborn Errors |
| D008664 | Metal Metabolism, Inborn Errors |
| D008659 | Metabolic Diseases |
| D009750 | Nutritional and Metabolic Diseases |