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Semiquantitative Scale for Assessing Brain MRI Abnormalities in Wilson Disease: A Validation Study
Petr Dusek1,2, Lukasz Smolinski3, Barbara Redzia-Ogrodnik4
1Department of Neurology and Center of Clinical Neuroscience, Charles University in Prague, First Faculty of Medicine and General University Hospital, Prague, Czech Republic.
Background:
MRI is a sensitive method for the assessment of brain abnormalities in Wilson disease, that is, T2 hyperintensities, T2 hypointensities, and atrophy, but a validated scoring system for the classification of radiological severity is lacking. The objective of this study was to develop and validate a brain MRI visual rating scale for Wilson disease.
Methods:
The proposed Wilson disease brain MRI severity scale consists of acute toxicity and chronic damage subscores from predefined structures. The former, calculated by summing scores of T2 hyperintensities (excluding cavitation), is likely to be partially reversible with treatment. The latter, representing the sum of scores of T2 hypointensities and brain atrophy, reflects pathology that is not readily reversible. Validation was performed on MRI scans acquired using 1.5T system from 39 Wilson disease patients examined at baseline and after 24 months on anticopper treatment. Intraclass correlation coefficients of 5 ratings from 3 raters were calculated. Temporal evolution of the MRI severity score and its association with clinical severity, assessed using the Unified Wilson Disease Rating Scale part III, was calculated.
Results:
Intrarater and interrater agreement were good (r > 0.93; P < 0.001; and r > 0.74; P < 0.001, respectively). In neurologic Wilson disease patients, the total MRI severity score improved over 2 years (P = 0.032), mainly because of reduced acute toxicity (P = 0.0015), whereas the chronic damage score deteriorated (P = 0.035). Unified Wilson Disease Rating Scale part III score was positively associated with chronic damage and total score at baseline (P = 0.005 and P = 0.003, respectively) and in month 24 (P < 0.001 and P = 0.001, respectively).
Conclusions:
The Wilson disease brain MRI severity scale is a simple, reliable, and valid instrument that allows semiquantitative assessment of radiological Wilson disease severity. © 2020 International Parkinson and Movement Disorder Society.
Insights
A new MRI severity scale for Wilson disease was developed and validated. This tool reliably assesses radiological severity, showing improvements in acute toxicity but worsening chronic damage over two years.
Area of Science:
- Neurology
- Radiology
- Medical Imaging
Background:
- Magnetic Resonance Imaging (MRI) is crucial for detecting brain abnormalities in Wilson disease, including T2 hyperintensities, T2 hypointensities, and atrophy.
- A validated scoring system for quantifying radiological severity in Wilson disease is currently lacking.
- This study aimed to address this gap by developing and validating a brain MRI visual rating scale specifically for Wilson disease.
Purpose of the Study:
- To develop and validate a brain MRI visual rating scale for assessing radiological severity in Wilson disease.
- To differentiate between reversible (acute toxicity) and non-reversible (chronic damage) MRI findings.
- To correlate MRI findings with clinical severity using the Unified Wilson Disease Rating Scale.
Main Methods:
- A novel Wilson disease brain MRI severity scale was created, incorporating subscores for acute toxicity (T2 hyperintensities) and chronic damage (T2 hypointensities and atrophy).
- The scale was validated using 1.5T MRI scans from 39 Wilson disease patients at baseline and after 24 months of anticopper treatment.
- Interrater and intrarater reliability were assessed, along with the temporal evolution of the MRI score and its association with clinical scores.
Main Results:
- The developed MRI severity scale demonstrated good intrarater and interrater agreement.
- In patients with neurologic Wilson disease, the total MRI severity score improved over two years, primarily due to reduced acute toxicity.
- The chronic damage score showed deterioration over the study period, and both total and chronic scores correlated positively with clinical severity.
Conclusions:
- The Wilson disease brain MRI severity scale is a simple, reliable, and valid tool for semiquantitative assessment of radiological Wilson disease.
- The scale allows for tracking treatment response and disease progression through distinct measures of acute and chronic brain changes.
- This validated scale can aid in standardizing the evaluation of brain involvement in Wilson disease and monitoring therapeutic interventions.
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