ENZYME REPLACEMENT THERAPY IN ADULT-ONSET GLYCOGENOSIS II: IS QUANTITATIVE MUSCLE MRI HELPFUL?

被引：26

作者：

Pichiecchio, A. ^{[1
]}

Poloni, G. U. ^{[1
]}

Ravaglia, S. ^{[2
]}

Ponzio, M. ^{[3
]}

Germani, G. ^{[1
]}

Maranzana, D. ^{[1
]}

Costa, A. ^{[2
]}

Repetto, A. ^{[2
]}

Tavazzi, E. ^{[2
]}

Danesino, C. ^{[4
]}

Moglia, A. ^{[2
]}

Bastianiello, S. ^{[1
]}

机构：

[1] Univ Pavia, IRCCS C Mondino Inst Neurol Fdn, Dept Neuroradiol, I-27100 Pavia, Italy

[2] Univ Pavia, IRCCS C Mondino Inst Neurol Fdn, Dept Neurol, I-27100 Pavia, Italy

[3] Univ Pavia, Dept Hlth Sci, Unit Med Stat & Epidemiol, I-27100 Pavia, Italy

[4] IRCCS S Matteo Hosp, San Giovanni Rotondo, Italy

来源：

MUSCLE & NERVE | 2009年 / 40卷 / 01期

关键词：

muscle MRI; glycogenosis II; Pompe disease; acid maltase deficiency; ERT; enzyme-replacement therapy; CONGENITAL MUSCULAR-DYSTROPHY; ACID ALPHA-GLUCOSIDASE; RIGID SPINE SYNDROME; BODY-COMPOSITION; POMPE-DISEASE; NEMALINE MYOPATHY; SKELETAL-MUSCLE; ADIPOSE-TISSUE; QUANTIFICATION; RECOMBINANT;

D O I：

10.1002/mus.21304

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Although it has been shown that muscle magnetic resonance imaging (MRI) improves the phenotypic characterization of patients with neuromuscular disorders and allows accurate quantification of muscle and adipose tissue distribution, to date quantitative MRI has not been used to assess the therapeutic response in clinical trials of neuromuscular diseases. We discuss quantitative MRI findings after a 6-month course of enzyme replacement therapy administered to nine patients with adult-onset glycogenosis II. Muscle Nerve 40: 122-125, 2009

引用

页码：122 / 125

页数：4

共 32 条

[1] MR quantification of muscle fatty replacement in McArdle's disease [J].