Document Detail


Correlation among subcortical white matter lesions, intelligence and CTG repeat expansion in classic myotonic dystrophy type 1.
MedLine Citation:
PMID:  18184345     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVES: To analyze the correlation among intelligence, brain magnetic resonance images (MRI) and genotype in classic myotonic dystrophy type 1 (DM1) patients. MATERIALS AND METHODS: Seventeen patients with classic DM1 were administered intelligence and neuropsychological tests and brain MRI focusing on a semi-quantitative rating scale of subcortical white matter lesions (WMLs). Statistical analysis was measured to evaluate the correlation among clinical manifestations, intelligence, brain MRI abnormalities, and CTG repeat expansion. RESULTS: There were statistically significant correlations between intelligence test and insular WMLs for all DM1 patients and between intelligence quotient and temporal WMLs for those patients with less than 400 of the CTG repeat size. We also documented that temporal WMLs were related to the disease course, and frontal WMLs were correlated with aging in all DM1 patients. However, a poor correlation was found among CTG repeat size and clinical pictures, neuropsychological impairments, and brain MRI abnormalities in all DM1 patients. CONCLUSION: These results suggest that subcortical WMLs are correlated with focal dementia in classic DM1 patients. Temporal and insular WMLs may be responsible for the global intellectual dysfunction of adult DM1 patients.
Authors:
H-C Kuo; Y-C Hsieh; H-M Wang; W-L Chuang; C-C Huang
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Acta neurologica Scandinavica     Volume:  117     ISSN:  1600-0404     ISO Abbreviation:  Acta Neurol. Scand.     Publication Date:  2008 Feb 
Date Detail:
Created Date:  2008-01-10     Completed Date:  2008-03-03     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0370336     Medline TA:  Acta Neurol Scand     Country:  Denmark    
Other Details:
Languages:  eng     Pagination:  101-7     Citation Subset:  IM    
Affiliation:
Department of Neurology, Chang Gung Memorial Hospital and College of Medicine, Chang Gung University, Taipei, Taiwan.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Brain / pathology*
Female
Humans
Intelligence / physiology*
Magnetic Resonance Imaging / methods
Male
Middle Aged
Myotonic Dystrophy* / genetics,  pathology,  physiopathology
Neuroglia / pathology*
Neuropsychological Tests / statistics & numerical data
Protein-Serine-Threonine Kinases / genetics*
Statistics, Nonparametric
Trinucleotide Repeat Expansion / genetics*
Chemical
Reg. No./Substance:
EC 2.7.1.-/myotonic dystrophy protein kinase; EC 2.7.11.1/Protein-Serine-Threonine Kinases

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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