Cognitive Impairment in Myotonic Dystrophy Type 1 Is Associated with White Matter Damage
- Caso Francesca
- Agosta Federica
- Peric Stojan
- Rakočević-Stojanović Vidosava
- Copetti Massimiliano
- Kostic Vladimir S.
- Filippi Massimo
DOIAbstract
To investigate grey (GM) and white matter (WM) abnormalities and their effects on cognitive and behavioral deficits in a large, phenotypically and genotypically well-characterized cohort of classic adult (aDM1, age at onset ≥20 years) or juvenile (jDM1, age at onset <20 years) patients with myotonic dystrophy type 1 (DM1).A case-control study including 51 DM1 patients (17 jDM1 and 34 aDM1) and 34 controls was conducted at an academic medical center. Clinical, cognitive and structural MRI evaluations were obtained. Quantitative assessments of regional GM volumes, WM hyperintensities (WMHs), and microstructural WM tract damage were performed. The association between structural brain damage and clinical and cognitive findings was assessed.DM1 ...
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