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
- 2015.1
- 수록면
- 331 - 338 (8page)
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초록· 키워드
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Background and Purpose Hyperkalemic periodic paralysis (hyperKPP) is a muscle sodium-ion channelopathy characterized by recurrent paralytic attacks. A proportion of afected individuals develop fxed or chronic progressive weakness that results in signifcant disability.
However, little is known about the pathology of hyperKPP-induced fxed weakness, including the pattern of muscle involvement. Te aim of this study was to characterize the patterns of muscle involvement in hyperKPP by whole-body magnetic resonance imaging (MRI).
Methods We performed whole-body muscle MRI in seven hyperKPP patients carrying the T704M mutation in the SCN4A skeletal sodium-channel gene. Muscle fat infltration, suggestive of chronic progressive myopathy, was analyzed qualitatively using a grading system and was quantifed by the two-point Dixon technique.
Results Whole-body muscle MRI analysis revealed muscle atrophy and fatty infltration in hyperKPP patients, especially in older individuals. Muscle involvement followed a selective pattern, primarily affecting the posterior compartment of the lower leg and anterior thigh muscles. The muscle fat fraction increased with patient age in the anterior thigh (r=0.669, p=0.009), in the deep posterior compartment of the lower leg (r=0.617, p=0.019), and in the superfcial posterior compartment of the lower leg (r=0.777, p=0.001).
Conclusions Our whole-body muscle MRI fndings provide evidence for chronic progressive myopathy in hyperKPP patients. Te reported data suggest that a selective pattern of muscle involvement—afecting the posterior compartment of the lower leg and the anterior thigh—is characteristic of chronic progressive myopathy in hyperKPP.
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참고문헌 (17)
참고문헌 신청
Amarteifio E / 2012 / Hyperkalemic periodic paralysis and permanent weakness: 3-T MR imaging depicts intracellular 23Na overload--initial results / Radiology 264 : 154 ~ 163
Bradley WG / 1990 / Progressive myopathy in hyperkalemic periodic paralysis / Arch Neurol 47 : 1013 ~ 1017
Charles G / 2013 / Characterization of hyperkalemic periodic paralysis: a survey of genetically diagnosed individuals / J Neurol 260 : 2606 ~ 2613
Hollingsworth KG / 2012 / Towards harmonization of protocols for MRI outcome measures in skeletal muscle studies: consensus recommendations from two TREAT-NMD NMR workshops /
Ma J / 2008 / Dixon techniques for water and fat imaging / J Magn Reson Imaging 28 : 543 ~ 558
Bradley WG / 1990 / Progressive myopathy in hyperkalemic periodic paralysis / Arch Neurol 47 : 1013 ~ 1017
Charles G / 2013 / Characterization of hyperkalemic periodic paralysis: a survey of genetically diagnosed individuals / J Neurol 260 : 2606 ~ 2613
Hollingsworth KG / 2012 / Towards harmonization of protocols for MRI outcome measures in skeletal muscle studies: consensus recommendations from two TREAT-NMD NMR workshops /
Ma J / 2008 / Dixon techniques for water and fat imaging / J Magn Reson Imaging 28 : 543 ~ 558
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