Academic Journal
Lesion distribution and substrate of white matter damage in myotonic dystrophy type 1: Comparison with multiple sclerosis
| العنوان: | Lesion distribution and substrate of white matter damage in myotonic dystrophy type 1: Comparison with multiple sclerosis |
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| المؤلفون: | Sara Leddy, Laura Serra, Davide Esposito, Camilla Vizzotto, Giovanni Giulietti, Gabriella Silvestri, Antonio Petrucci, Giovanni Meola, Leonardo Lopiano, Mara Cercignani, Marco Bozzali |
| المصدر: | NeuroImage: Clinical, Vol 29, Iss , Pp 102562- (2021) |
| بيانات النشر: | Elsevier, 2021. |
| سنة النشر: | 2021 |
| المجموعة: | LCC:Computer applications to medicine. Medical informatics LCC:Neurology. Diseases of the nervous system |
| مصطلحات موضوعية: | DM1, qMT, MS, Lesion, White matter, MRI, Computer applications to medicine. Medical informatics, R858-859.7, Neurology. Diseases of the nervous system, RC346-429 |
| الوصف: | Myotonic Dystrophy type 1 (DM1) is an autosomal dominant condition caused by expansion of the CTG triplet repeats within the myotonic dystrophy protein of the kinase (DMPK) gene. The central nervous system is involved in the disease, with multiple symptoms including cognitive impairment. A typical feature of DM1 is the presence of widespread white matter (WM) lesions, whose total volume is associated with CTG triplet expansion. The aim of this study was to characterize the distribution and pathological substrate of these lesions as well as the normal appearing WM (NAWM) using quantitative magnetization transfer (qMT) MRI, and comparing data from DM1 patients with those from patients with multiple sclerosis (MS). Twenty-eight patients with DM1, 29 patients with relapsing-remitting MS, and 15 healthy controls had an MRI scan, including conventional and qMT imaging. The average pool size ratio (F), a proxy of myelination, was computed within lesions and NAWM for every participant. The lesion masks were warped into MNI space and lesion probability maps were obtained for each patient group. The lesion distribution, total lesion load and the tissue-specific mean F were compared between groups. The supratentorial distribution of lesions was similar in the 2 patient groups, although mean lesion volume was higher in MS than DM1. DM1 presented higher prevalence of anterior temporal lobe lesions, but none in the cerebellum and brainstem. Significantly reduced F values were found within DM1 lesions, suggesting a loss of myelin density. While F was reduced in the NAWM of MS patients, it did not differ between DM1 and controls. Our results provide further evidence for a need to compare histology and imaging using new MRI techniques in DM1 patients, in order to further our understanding of the underlying disease process contributing to WM disease. |
| نوع الوثيقة: | article |
| وصف الملف: | electronic resource |
| اللغة: | English |
| تدمد: | 2213-1582 90659759 |
| Relation: | http://www.sciencedirect.com/science/article/pii/S2213158221000061; https://doaj.org/toc/2213-1582 |
| DOI: | 10.1016/j.nicl.2021.102562 |
| URL الوصول: | https://doaj.org/article/f7ee9065975945bf82dbefba6af979f6 |
| رقم الانضمام: | edsdoj.f7ee9065975945bf82dbefba6af979f6 |
| قاعدة البيانات: | Directory of Open Access Journals |
Full Text Finder | تدمد: | 22131582 90659759 |
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| DOI: | 10.1016/j.nicl.2021.102562 |