التفاصيل البيبلوغرافية
| العنوان: |
Clinical Significance of Diffusion Tensor Imaging in Metachromatic Leukodystrophy |
| المؤلفون: |
Amedick, Lucas Bastian, Martin, Pascal, Beschle, Judith, Strölin, Manuel, Wilke, Marko, Wolf, Nicole, Pouwels, Petra, Hagberg, Gisela, Klose, Uwe, Naegele, Thomas, Kraegeloh-Mann, Ingeborg, Groeschel, Samuel |
| المساهمون: |
Takeda Pharma AG, German Research Foundation |
| المصدر: |
Neuropediatrics ; volume 54, issue 04, page 244-252 ; ISSN 0174-304X 1439-1899 |
| بيانات النشر: |
Georg Thieme Verlag KG |
| سنة النشر: |
2023 |
| الوصف: |
Background Metachromatic leukodystrophy (MLD) is a lysosomal enzyme deficiency disorder leading to progressive demyelination and, consecutively, to cognitive and motor decline. Brain magnetic resonance imaging (MRI) can detect affected white matter as T2 hyperintense areas but cannot quantify the gradual microstructural process of demyelination more accurately. Our study aimed to investigate the value of routine MR diffusion tensor imaging in assessing disease progression. Methods MR diffusion parameters (apparent diffusion coefficient [ADC] and fractional anisotropy [FA]) were in the frontal white matter, central region (CR), and posterior limb of the internal capsule in 111 MR datasets from a natural history study of 83 patients (age: 0.5–39.9 years; 35 late-infantile, 45 juvenile, 3 adult, with clinical diffusion sequences of different scanner manufacturers) as well as 120 controls. Results were correlated with clinical parameters reflecting motor and cognitive function. Results ADC values increase and FA values decrease depending on disease stage/severity. They show region-specific correlations with clinical parameters of motor and cognitive symptoms, respectively. Higher ADC levels in CR at diagnosis predicted a disease course with more rapid motor deterioration in juvenile MLD patients. In highly organized tissues such as the corticospinal tract, in particular, diffusion MR parameters were highly sensitive to MLD-associated changes and did not correlate with the visual quantification of T2 hyperintensities. Conclusion Our results show that diffusion MRI can deliver valuable, robust, clinically meaningful, and easily obtainable/accessible/available parameters in the assessment of prognosis and progression of MLD. Therefore, it provides additional quantifiable information to established methods such as T2 hyperintensity. |
| نوع الوثيقة: |
article in journal/newspaper |
| اللغة: |
English |
| DOI: |
10.1055/a-2073-4178 |
| DOI: |
10.1055/a-2073-4178.pdf |
| الإتاحة: |
https://doi.org/10.1055/a-2073-4178Test |
| حقوق: |
https://creativecommons.org/licenses/by-nc-nd/4.0Test/ |
| رقم الانضمام: |
edsbas.E4EF5E6C |
| قاعدة البيانات: |
BASE |
