Radboud Centre for Mitochondrial Medicine Pediatric MRI score

We developed the first user-friendly, semi-quantitative, and quick-to-perform Radboud Centre for Mitochondrial Medicine Pediatric MRI score (RCMM-PMRIS), focusing on the six most commonly described neuroimaging abnormalities in the literature. The RCMM-PMRIS was validated through individual review o...

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Published inMitochondrion Vol. 32; pp. 36 - 41
Main Authors Wong, Sheila Suet-Na, Goraj, Bozena, Fung, Cheuk-Wing, Vister, Jeroen, de Boer, Lonneke, Koene, Saskia, Smeitink, Jan
Format Journal Article
LanguageEnglish
Published Netherlands 01.01.2017
Subjects
Adolescent
Brain - diagnostic imaging
Brain - pathology
Child
Child, Preschool
Female
Humans
Infant
Infant, Newborn
Magnetic Resonance Imaging - methods
Male
Mitochondrial Diseases - diagnostic imaging
Severity of Illness Index
End point
Mitochondrial disorders
Magnetic resonance imaging
Mitochondrial disease
Outcome measure
Online AccessGet full text
ISSN1567-7249
1872-8278
DOI10.1016/j.mito.2016.11.008

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Summary:We developed the first user-friendly, semi-quantitative, and quick-to-perform Radboud Centre for Mitochondrial Medicine Pediatric MRI score (RCMM-PMRIS), focusing on the six most commonly described neuroimaging abnormalities in the literature. The RCMM-PMRIS was validated through individual review of 30 sets of brain MRI studies in 24 patients with genetically confirmed mitochondrial disorders by six raters. The application of RCMM-PMRIS can help to define the extent of the brain involvement and therefore to assess the radiological mitochondrial disease severity, to monitor disease progression and consequently to act as an outcome measure for treatment effects in patients with mitochondrial disease.
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ISSN:1567-7249
1872-8278
DOI:10.1016/j.mito.2016.11.008