지원사업
학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
커뮤니티
연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2017.7
- 수록면
- 807 - 815 (9page)
- DOI
- 10.3349/ymj.2017.58.4.807
이용수
초록· 키워드
오류제보하기
Purpose: Myotonic dystrophy type 1 (DM1) is characterized by progressive muscular weakness with symptoms caused by involvementof the brain. The aim of this study was to delineate global changes in cortical thickness and white matter integrity in patients with DM1, compared to age-matched healthy controls, and in brain areas highly correlated with CTG repeat size.
Materials and Methods: Cortical thickness and white matter integrity were compared in nine adult DM1 patients and age matched healthy controls using T1-weighted and diffusion tensor imaging. The patients’ intelligence quotient (IQ) and CTG repeatsize were measured in each individual.
Results: Cortical thickness was significantly reduced in the frontal, temporal, and occipital cortices, while tract-based spatial statisticsshowed decreased diffusion metrics in widespread areas, including the bilateral orbitofrontal, anterior frontal, insular, externalcapsule, and occipital cortices in DM1 patients, compared to controls. Additionally, thickness was negatively correlated with the number of CTG repeats in those areas. White matter integrity was negatively correlated with CTG repeats in the left entorhinal,anterior corona radiata, orbitofrontal, and lateral occipital areas. No statistically significant correlation was found between IQ scores and the size of CTG repeats.
Conclusion: Our results suggest that DM1 is associated with wide distributions of network changes in both gray and white matter. Some of areas related to cognition showed significant correlations with CTG repeats.
목차
등록된 정보가 없습니다.
참고문헌 (41)
참고문헌 신청
Brook JD / 1992 / Molecular basis of myotonic dystrophy: expansion of a trinucleotide (CTG) repeat at the 3’ end of a transcript encoding a protein kinase family member / Cell 69:385
Huber SJ / 1989 / Magnetic resonance imaging and clinical correlates of intellectual impairment in myotonic dystrophy / Arch Neurol 46:536~540
Meola G / 1999 / Reduced cerebral blood flow and impaired visual-spatial function in proximal myotonic myopathy / Neurology 53:1042~1050
Modoni A / 2004 / Characterization of the pattern of cognitive impairment in myotonic dystrophy type 1 / Arch Neurol 61:1943~1947
Meola G / 2003 / Executive dysfunction and avoidant personality trait in myotonic dystrophy type 1 (DM-1) and in proximal myotonic myopathy (PROMM/DM-2) / Neuromuscul Disord 13:813~821
Huber SJ / 1989 / Magnetic resonance imaging and clinical correlates of intellectual impairment in myotonic dystrophy / Arch Neurol 46:536~540
Meola G / 1999 / Reduced cerebral blood flow and impaired visual-spatial function in proximal myotonic myopathy / Neurology 53:1042~1050
Modoni A / 2004 / Characterization of the pattern of cognitive impairment in myotonic dystrophy type 1 / Arch Neurol 61:1943~1947
Meola G / 2003 / Executive dysfunction and avoidant personality trait in myotonic dystrophy type 1 (DM-1) and in proximal myotonic myopathy (PROMM/DM-2) / Neuromuscul Disord 13:813~821
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