Metachromatic leukodystrophy: a scoring system for brain MR imaging observations.
AJNR Am J Neuroradiol
; 30(10): 1893-7, 2009 Nov.
Article
em En
| MEDLINE
| ID: mdl-19797797
ABSTRACT
BACKGROUND AND PURPOSE:
Metachromatic leukodystrophy (MLD) is a devastating demyelinating disease for which novel therapies are being tested. We hypothesized that MR imaging of brain lesion involvement in MLD could be quantified along a scale. MATERIALS ANDMETHODS:
Thirty-four brain MR images in 28 patients with proved biochemical and genetic defects for MLD were reviewed 10 patients with late infantile, 16 patients with juvenile, and 2 patients with adult MLD. All MR images were reviewed by experienced neuroradiologists and neurologists (2 readers in Germany, 2 readers in the United States) for global disease burden, as seen on the T2 and fluid-attenuated inversion recovery images. A visual scoring method was based on a point system (range, 0-34) derived from the location of white matter involvement and the presence of global atrophy, analogous to the scoring system developed for adrenoleukodystrophy. The readers were blinded to the neurologic findings.RESULTS:
Thirty-three of 34 MR images showed confluent T2 hyperintensities of white matter. The inter-rater reliability coefficient was 0.988. Scores between readers were within 2 points of each other. Serial MR imaging studies in 6 patients showed significant progressive disease in 3 patients (initial score average, 4; mean follow-up, 24.3) and no change or 1 point progression in 3 patients (initial score average, 12; mean follow-up, 12.66). Projection fibers and the cerebellum tended to be involved only in advanced stages of disease.CONCLUSIONS:
The MLD MR severity scoring method can be used to provide a measure of brain MR imaging involvement in MLD patients.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Índice de Gravidade de Doença
/
Encéfalo
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Imageamento por Ressonância Magnética
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Leucodistrofia Metacromática
Limite:
Adolescent
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Adult
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Child
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Child, preschool
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Humans
Idioma:
En
Ano de publicação:
2009
Tipo de documento:
Article