Toward a Better Understanding of Brain Lesions during Metachromatic Leukodystrophy Evolution
| Autor: | P Aubourg, Catherine Adamsbaum, C. Lazarus, C. Bellesme, C Sevin, A. Martin |
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| Rok vydání: | 2012 |
| Předmět: |
Male
medicine.medical_specialty Wilcoxon signed-rank test Sensitivity and Specificity Pediatrics 030218 nuclear medicine & medical imaging 03 medical and health sciences 0302 clinical medicine Humans Medicine Radiology Nuclear Medicine and imaging Statistical analysis Mri brain business.industry T2 hypointensity Brain Reproducibility of Results Leukodystrophy Metachromatic medicine.disease Magnetic Resonance Imaging Hyperintensity Metachromatic leukodystrophy Child Preschool Disease Progression Brain lesions Female Neurology (clinical) Radiology business Neuroscience 030217 neurology & neurosurgery Student's t-test |
| Zdroj: | American Journal of Neuroradiology; Vol 33 AJNR Am J Neuroradiol American Journal of Neuroradiology |
| ISSN: | 1936-959X 0195-6108 |
| DOI: | 10.3174/ajnr.a3038 |
| Popis: | BACKGROUND AND PURPOSE: The prospect of new therapies in MLD stresses the need to refine the indications for treatment. The aim of this study was, therefore, to perform a detailed analysis of MRI brain lesions at diagnosis and follow-up, to better understand the natural history of MLD. MATERIAL AND METHODS: This retrospective case-control study (2005–2010) looked at 13 patients with MLD (2–5 years of age) with 28 MRIs (mean follow-up, 2 years), compared with 39 age- and sex-matched controls. All MRIs were evaluated qualitatively and semiquantitatively. The Student t test, Wilcoxon signed rank test, and Pearson correlation were used for statistical analysis (P < .05). RESULTS: In addition to diffuse symmetric supratentorial WM T2 hyperintensities with a tigroid pattern (70%) and T2 hyperintensities in the CC (100%) and internal capsules (46%), we found significant GM abnormalities such as thalamic T2 hypointensity (92%), thalamic (23%, P < .05, EJ) and caudate nuclei (23%, P < .05, EJ) atrophy, and cerebellar atrophy without WM involvement (15%). The pattern of splenium involvement progression was misleading, with initially diffuse high signal intensity, which later became curvilinear before finally progressing to atrophy (23%, P < .05; EJ). This should not be mistaken for a disease regression. Spectroscopy confirmed a decrease in the NAA/Cr ratio, an increase in the Cho/Cr ratio and in myo-inositol, and a lactate resonance. CONCLUSIONS: Thalamic changes may be a common finding in MLD, raising the prospect of primary GM lesions. This may prove important when evaluating the efficacy of new treatments. |
| Databáze: | OpenAIRE |
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