Clinical Value of DWI-ADC Matching in the Short-term Prognosis of Wilson's Disease

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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Key information

Conditions

Hepatolenticular Degeneration; Wilson Basal Ganglia Diseases Brain Diseases Brain Diseases, Metabolic Brain Diseases, Metabolic, Inborn Central Nervous System Diseases Congenital, Hereditary, and Neonatal Diseases and Abnormalities Digestive System Diseases Genetic Diseases, Inborn Hepatolenticular Degeneration Heredodegenerative Disorders, Nervous System Liver Diseases Metabolic Diseases Metabolism, Inborn Errors Metal Metabolism, Inborn Errors Movement Disorders Nervous System Diseases Neurodegenerative Diseases Nutritional and Metabolic Diseases

Sex eligibility

All sexes

Study type

Observational

Primary location

The affiliated hospital of Institute of Neurology in Anhui University of Chinese Medicine

Hefei, Anhui, 230001, China

Who can participate

Healthy volunteers accepted: No

Only the study team can determine whether someone qualifies for participation.

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 time；
Patients who accept de-copper treatment in hospitalization for 4 weeks or more.

Exclusion criteria

Patients who only with liver injury;
Severe complications;
Combined with other diseases.

Treatment and study plan

Primary outcomes

UWDRS scales

Time frame: Before and 4 weeks after treatment

It is used to evaluate the changes of patients' nervous system symptoms
24h urine copper

Time frame: Before and 4 weeks after treatment

used to evaluate the changes of patients' nervous system symptoms

Secondary outcomes

Semi-quantitative brain MRI score

Time frame: Before de-copper treatment

It is used to evaluate the changes of patients' nervous system symptoms
ALT, AST and other liver function test

Time frame: Before and 4 weeks after treatment

used to evaluate the changes of patients' nervous system symptoms

Sponsors and collaborators

Lead sponsor

Hospital Affiliated to the Institute of Neurology, Anhui University of Chinese Medicine

Other

Registry information

Important dates

Study start: 2024
Primary completion: 2025
Study completion: 2026
First posted: Jan 9, 2024
Registry last updated: Jan 9, 2024

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This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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