Structural and functional abnormalities of the brain in myotonic dystrophy type 1 (MD1) and 2 (MD2)
R Roebling, Y.G Weber, J Kassubek, S Hoffmann, A Rosenbohm, J Gostomzyk, M Wolf, W Vogel, P Steinbach, H Walter, S.N Reske, F Lehmann-Horn, F Mottaghy, H Lerche · Aktuelle Neurologie · 2008 · Heft 09
Bibliografische Angaben
Zusammenfassung
Myotonic dystrophy type 1 and 2 (DM1/DM2) are slowly progressive multisystemic diseases. Core features are myotonia, muscle weakness and atrophy, with a more benign course in DM2. Further clinical findings are cardiac arrhythmia, cataracts, endocrine changes and cognitive deficits. In the present study, neuropsychological deficits were characterized and correlated to brain imaging in 20 DM1 and 9 DM2 patients compared to healthy controls using a broad spectrum of neuropsychological tests, cerebral magnetic resonance imaging (cMRI) and 18F-deoxy-glucose positron emission tomography (FDG-PET). Deficits in visual-s…
