Cognitive impairment in adult myotonic dystrophies: a longitudinal study.
The clinical relevance and extent of cognitive impairment in adult myotonic dystrophy type 1 (DM1) and 2 (DM2) is still unclear. The aim of this study was to determine whether previously reported cognitive abnormalities progress over time and if this occurs in DM2 as it does in DM1. Fifty-six patients with DM1 and 29 patients with DM2 were subjected to muscle strength assessment, and to a complete battery of neuropsychological tests. Repeated assessment was performed in 20 DM1 and 13 DM2 over time (DM1 mean follow-up: 7.3+/-2.7 years; DM2 mean follow- up: 9.5+/-2.4 years). Muscle strength and test scores for frontal lobe functions worsened significantly over time (p<0.01), in both DM1 and DM2. DM2 is a progressive muscle disorder, although less severe than DM1. In both DM1 and DM2 frontal cognitive impairment (attentional) worsens over time but does not extend to additional areas of cognition.
Sansone
V
V
Department of Neurology, University of Milan, IRCCS Policlinico San Donato, Via Morandi 30, I-20097 San Donato Milanese, Italy.
Gandossini
S
S
Cotelli
M
M
Calabria
M
M
Zanetti
O
O
Meola
G
G
D000328:Adult; D000704:Analysis of Variance; D001288:Attention / Q000502:physiology; D003072:Cognition Disorders / Q000209:etiology*; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D008568:Memory; D008875:Middle Aged; D053580:Muscle Strength / Q000502:physiology; D009223:Myotonic Dystrophy / Q000145:classification / Q000150:complications*; D009483:Neuropsychological Tests; D011340:Problem Solving / Q000502:physiology; D018709:Statistics, Nonparametric