Cognitive and neuroradiological findings in myotonic dystrophy
Sinforiani, E.; Sandrini, G.; Martelli, A.; Mauri, M.; Uggetti, C.; Bono, G.; Nappi, G.
Functional Neurology 6(4): 377-384
1991
ISSN/ISBN: 0393-5264 PMID: 1810838 Document Number: 381983
Cognitive functions were investigated in 37 patients with myotonic dystrophy (MD) and correlated with clinical and neuroradiological variables. The whole cognitive performance was at a low-average level; in about 1/3 of the subjects, in fact, the scores at the neuropsychological tests were below the normal range. There was a consistent trend for patients with inheritance on maternal side to perform worse on Wechsler verbal score and to present cerebral atrophy. In 7 out of 12 subjects focal white matter lesions were found at nuclear magnetic resonance. The significance of these findings and its relation to cognitive performance are discussed.