Cognition in myotonic dystrophy type 1: a 5-year follow-up study.
Studies on cognitive decline in myotonic dystrophy type 1 (DM1) are characterized by conflicting results. The purpose of the present study was to analyse possible decline in classical/adult onset DM1 at a 5-year follow-up and to explore the correlation with disease-related and demographic factors.



Patients with DM1 (n = 37) were examined with a comprehensive neuropsychological test battery yielding measures on memory, attention, verbal, visuospatial and executive functions. Assessment of muscle impairment and CTG repeat expansion size was performed.



A majority of the participants (65%) performed worse at follow-up. Compared to normative data, patients scored significantly worse on tests measuring memory, attention, visuospatial construction and verbal ability. Neither CTG repeat size nor muscle impairment related to cognitive decline. However, age at onset and disease duration were correlated with the number of tests in which performance was below 1 SD at both baseline and follow-up examination.



Measurements show that classical/adult onset DM1 is characterized by cognitive decline. Both earlier onset and longer duration of the disease are indicative of more cognitive deficits.
Department of Psychology, University of Gothenburg, Gothenburg, Sweden.|Neuromuscular Centre, Department of Neurology, Sahlgrenska University Hospital, Gothenburg, Sweden.
S
S
Winblad
Department of Clinical Genetics, Sahlgrenska University Hospital, Gothenburg, Sweden.
L
L
Samuelsson
Neuromuscular Centre, Department of Neurology, Sahlgrenska University Hospital, Gothenburg, Sweden.|Department of Clinical Genetics, Sahlgrenska University Hospital, Gothenburg, Sweden.
C
C
Lindberg
Department of Biomedical Sciences for Health, IRCCS Policlinico San Donato, University of Milan, San Donato Milanese, Italy.
G
G
Meola
D000328:Adult; D001288:Attention; D003071:Cognition; D060825:Cognitive Dysfunction; D018450:Disease Progression; D056344:Executive Function; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008568:Memory; D000073216:Mental Status and Dementia Tests; D008875:Middle Aged; D009135:Muscular Diseases / Q000453:epidemiology / Q000209:etiology; D009223:Myotonic Dystrophy / Q000453:epidemiology / Q000235:genetics / Q000523:psychology*; D012959:Socioeconomic Factors; D013028:Space Perception; D019680:Trinucleotide Repeat Expansion; D055815:Young Adult
cognition; cognitive decline; myotonic dystrophy; neuromuscular