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学科主题: 神经病学
题名:
dysferlinopathy 患者大腿骨骼肌 MRI 改变及其与临床表现的相关性
其他题名: Muscle magnetic resonance imaging changes and relationship with clinical symptoms in patients with dysferlinopathy
作者: 金苏芹1; 张巍1; 吕鹤1; 柳青1; 宋书娟1; 杜婧1; 肖江喜1; 王朝霞1; 袁云1
关键词: 肌营养不良,肢带型 ; ; 肌,骨骼 ; 水肿 ; 磁共振成像 ; Muscular dystrophies,limb-girdle ; Leg ; Muscle,skeletal ; Edema ; Magnetic resonance imaging
刊名: 中华神经科杂志
发表日期: 2014
DOI: 10.3760/cma.j.issn.1006-7876.2014.06.013
期: 6, 页:412-417
收录类别: 中国科技核心期刊 ; CSCD
文章类型: Journal Article
摘要: 目的:探讨dysferlinopathy患者大腿肌肉水肿及脂肪化规律及其与临床表现之间的关系。方法收集2005-2013年就诊于北京大学第一医院的32例dysferlinopathy患者,包括13例肢带型肌营养不良2B型(limb girdle muscular dystrophy type 2B,LGMD2B)、13例Miyoshi肌病、4例近远端肌病及2例高肌酸激酶血症患者。对患者进行改良Gardner-Medwin and Walton ( GM-W)评分和大腿肌肉MRI检查,对MRI的脂肪化和水肿改变进行评分。计算LGMD2B和Miyoshi肌病患者在发病年龄、病程、GM-W评分、肌肉脂肪化和水肿评分以及分布模式之间的差异,并分析所有患者大腿肌肉脂肪化和水肿总评分与其病程、GM-W评分之间的相关性。结果所有患者的GM-W中位数评分为4.00(2.00,5.00)分,其中LGMD2B为4.00(3.00,4.50)分,Miyoshi肌病为4.00(2.00,5.00)分。30例患者(93.75%)出现肌肉脂肪化,在LGMD2B和Miyoshi肌病患者中分布模式相同,骨骼肌脂肪化平均总评分为(28.20±12.86)分,其中 LGMD2B 为(28.50±13.03)分,Miyoshi 肌病为(29.00±12.63)分。26例患者(81.25%)存在肌肉水肿,在LGMD2B和Miyoshi肌病患者中分布模式相同,骨骼肌水肿平均总评分为(18.36±13.60)分。其中LGMD2B为(22.88±11.59)分,Miyoshi 肌病为(16.77±13.80)分。两组患者在发病年龄、病程、GM-W评分、肌水肿和脂肪化评分之间差异无统计学意义。所有患者的肌肉脂肪化总评分与其GM-W评分( rs =0.737,P=0.000)和病程( rs =0.637, P=0.000)均呈正相关。结论大腿肌肉脂肪化和水肿均是dysferlinopathy 患者常见的MRI改变,主要临床亚型具有类似的影像学改变。肌肉脂肪化可用于该病的进展评估。 Objective To investigate the characteristics of muscle edema and fatty infiltration in thighs and relationship with clinical symptoms in Chinese patients with different phenotypes of dysferlinopathy.Methods A total of 32 patients were enrolled , including 13 limb-girdle muscular dystrophy 2B (LGMD2B), 13 Miyoshi myopathy (MM), 4 proximodistal myopathy and 2 hyper-creatine-kinase-emia.Clinical symptoms were evaluated using modified Gardner-Medwin and Walton ( GM-W) score.Muscle MRI was performed in thighs to observe fatty infiltration and edema.We then compared the age of onset , disease duration, GM-W score, muscle edema and muscle fatty infiltration between LGMD 2B and MM groups,and the relationship of muscle edema score and fatty infiltration score with disease duration and GM-W score in all patients.Results The median GM-W score was 4.00 (2.00,5.00) in all patients, 4.00 (3.00,4.50)in LGMD2B and 4.00(2.00,5.00)in MM, respectively.Muscle fatty infiltration appeared in 30 cases (93.75%), with the same pattern in LGMD2B and MM.The mean fatty infiltration score was 28.20 ±12.86 in all patients, 28.50 ±13.03 in LGMD2B and 29.00 ±12.63 in MM, respectively.Muscle edema appeared in 26 cases (81.25%) with same pattern in LGMB2B and MM.The mean edema score was 18.36 ±13.60 in all patients, 22.88 ±11.59 in LGMD2B and 16.77 ±13.80 in MM.The age of onset , disease duration, GM-W score, muscle fatty infiltration and edema score were not significantly different between LGMD2B and MM patients.Muscle fatty infiltration score significantly correlated with GM-W score (rs=0.737,P=0.000) and disease duration (rs=0.637,P=0.000).Conclusions Fatty infiltration and edema in thigh muscles are very common in patients with dysferlinopathy , with similar radiological changes in main subtypes.The muscle fatty infiltration can be used as a predictor of disease progression.
语种: 中文
原文出处: 查看原文
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内容类型: 期刊论文
URI标识: http://ir.bjmu.edu.cn/handle/400002259/41039
Appears in Collections:北京大学第一临床医学院_神经内科_期刊论文

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作者单位: 1.100034,北京大学第一医院神经内科
2.太原市中心医院神经内科
3.北京大学医学部医学遗传学系
4.北京大学第一医院医学影像科
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