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ERIC Number: EJ861491
Record Type: Journal
Publication Date: 2009-Dec
Pages: 9
Abstractor: As Provided
Reference Count: 0
ISBN: N/A
ISSN: ISSN-0012-1622
Cognition and Adaptive Skills in Myotonic Dystrophy Type 1: A Study of 55 Individuals with Congenital and Childhood Forms
Ekstrom, Anne-Berit; Hakenas-Plate, Louise; Tulinius, Mar; Wentz, Elisabet
Developmental Medicine & Child Neurology, v51 n12 p982-990 Dec 2009
Aims: To investigate cognitive abilities and adaptive skills in children and adolescents with myotonic dystrophy type 1 (DM1) and correlate the findings to the cytosine-thymine-guanine (CTG) repeat expansion size. Method: Cognitive level was assessed in 55 children and adolescents with DM1 (31 males, 24 females; mean age 12y 1mo, SD 5y 1mo; range 2y 7mo-21y 5mo) divided into the following categories: severe congenital DM1 (n = 19), mild congenital DM1 (n = 18), and childhood DM1 (n = 18). The Griffiths Mental Developmental Scale, the Wechsler Scales, and the Vineland Adaptive Behavior Scales (VABS) for adaptive skills were used for this purpose. Results: Learning disability was found in 95% of the severe congenital group, 83% of the mild congenital group, and 89% of the childhood DM1 group. The more severe the form of DM1, the lower the full-scale IQ (FSIQ; r[subscript s] = 0.28, p = 0.044). The individuals with severe congenital and childhood DM1 had a significantly higher verbal IQ than performance IQ (severe congenital: mean difference 5.7, SD 5.7, p = 0.008; childhood DM1: mean difference 9.8, SD 18.0, p = 0.038). CTG repeat expansion correlated negatively with FSIQ (r[subscript s] = -0.63, p less than 0.006). Almost all participants showed poor results on the VABS. There was a positive relationship between cognitive level and adaptive skills in the mild congenital (r[subscript s] = 0.95, p less than 0.01) and childhood DM1 groups (r[subscript s] = 0.92, p less than 0.01). Interpretation: Children and adolescents with DM1 exhibit significant cognitive and adaptive problems.
Wiley-Blackwell. 350 Main Street, Malden, MA 02148. Tel: 800-835-6770; Tel: 781-388-8598; Fax: 781-388-8232; e-mail: cs-journals@wiley.com; Web site: http://www.wiley.com/WileyCDA/
Publication Type: Journal Articles; Reports - Research
Education Level: N/A
Audience: N/A
Language: English
Sponsor: N/A
Authoring Institution: N/A