Clinical trial NCT06196931: Clinical Value of DWI-ADC Matching in the Short-term Prognosis of Wilson's Disease
Official title: Clinical Value of DWI-ADC Matching in the Short-term Prognosis of Wilson's Disease
Study type: OBSERVATIONAL
Overall status: NOT_YET_RECRUITING
Conditions: Hepatolenticular Degeneration; Wilson
Keywords: DWI-ADC matching
Wilson disease
clinical value
short-term prognosis
Lead sponsor: Hospital Affiliated to the Institute of Neurology, Anhui University of Chinese Medicine
Sponsor class: OTHER
First posted: 2024-01-09
Enrollment: 200
Sex: ALL
Standard ages: CHILD
ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: COHORT
Time perspective: RETROSPECTIVE
Sampling method: NON_PROBABILITY_SAMPLE
Study population: 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.

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.


Eligibility criteria: 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.

Primary outcomes:
measure: UWDRS scales
timeFrame: Before and 4 weeks after treatment
measure: 24h urine copper
timeFrame: Before and 4 weeks after treatment
Secondary outcomes:
measure: Semi-quantitative brain MRI score
timeFrame: Before de-copper treatment
measure: ALT, AST and other liver function test
timeFrame: Before and 4 weeks after treatment
Other outcomes:
Locations:
city: Hefei
country: China
facility: The affiliated hospital of Institute of Neurology in Anhui University of Chinese Medicine
state: Anhui
Study officials:
References: