A weighted cranial diffusion-weighted imaging scale for Wilson's disease

Area of Science:

• Neurology
• Radiology
• Medical Imaging

Background:

• Wilson's disease (WD) is a genetic disorder causing copper accumulation.
• Neurological symptoms in WD require accurate assessment tools.
• Cranial magnetic resonance imaging (MRI), particularly diffusion-weighted imaging (DWI), shows acute brain injuries in WD.

Purpose of the Study:

• To develop a weighted cranial DWI scale for assessing neurological symptoms in WD patients.
• To correlate the developed scale with clinical outcomes, including deterioration and readmission rates.
• To improve prognostic prediction and guide treatment management in WD.

Main Methods:

• 123 WD patients underwent 1.5 T-MRI.
• An imaging score was calculated based on T1, T2, fluid-attenuation inversion recovery, and DWI sequences.
• Consensus weighting was applied based on symptoms and treatment response.

Main Results:

• DWI hyperintensity in the putamen predicted deterioration during de-copper therapy (OR=8.656).
• DWI hyperintensity in the midbrain and corpus callosum predicted readmission (OR=3.818 and OR=2.654, respectively).
• The consensus scoring system showed higher correlations with deterioration and readmission compared to the original system.

Conclusions:

• The consensus semi-quantitative scoring system for cranial MRI improves prediction of WD patient outcomes.
• This scale can guide medication, healthcare management, and prognosis prediction.
• Each point increase in the score correlates with a 5.2% increase in exacerbation risk and a 4.3% increase in 6-month readmission risk.