Semiquantitative Scale for Assessing Brain MRI Abnormalities in Wilson Disease: A Validation Study
| Autor: | Tomasz Litwin, Petr Dusek, Barbara Redzia-Ogrodnik, Marta Skowrońska, Marek Gołębiowski, Lukasz Smolinski, Anna Członkowska, A. Poujois, Chloé Laurencin, Iwona Jastrzebska-Kurkowska |
|---|---|
| Rok vydání: | 2019 |
| Předmět: |
0301 basic medicine
medicine.medical_specialty Intraclass correlation Disease 03 medical and health sciences 0302 clinical medicine Atrophy Hepatolenticular Degeneration Rating scale Brain mri Medicine Humans Neuroradiology Brain Diseases business.industry Brain Reproducibility of Results medicine.disease Magnetic Resonance Imaging Hyperintensity Inter-rater reliability 030104 developmental biology Neurology Neurology (clinical) Radiology business 030217 neurology & neurosurgery |
| Zdroj: | Movement disorders : official journal of the Movement Disorder SocietyReferences. 35(6) |
| ISSN: | 1531-8257 |
| Popis: | 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 0.74; P |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|
Plný text