1型强直性肌营养不良中的肌肉残疾和器官损害
[Muscular disability and organ impairments in myotonic dystrophy type 1].
作者信息
Pruna L, Machado F, Louis L, Vassé G, Kaminsky P
机构信息
Médecine interne orientée vers les maladies orphelines et systémiques, CHU de Nancy, hôpitaux de Brabois, rue du Morvan, 54511 Vandœuvre cedex, France.
出版信息
Rev Neurol (Paris). 2011 Jan;167(1):23-8. doi: 10.1016/j.neurol.2010.07.026. Epub 2010 Oct 8.
BACKGROUND
Myotonic dystrophy (DM1) is a multisystemic disorder characterized by myotonic muscular weakness, and numerous organ impairments, especially cardiac and respiratory disorders. The goal of this study was to evaluate in DM1 patients the relationships between a new muscular disability scale, the motor function measure (MFM), and functional measurements of organ involvements.
PATIENTS AND METHODS
The MFM and MDRS, as well as spirometry, blood gases, echocardiography, electrocardiogram, and ophthalomological examination were performed in 69 consecutive DM1 patients.
RESULTS
Significant relationships were found between MDRS and MFM (p < 0.001) as well as between both and age and BMI of the patients. Patients with total MFM below 76 had a higher risk of respiratory insufficiency, conduction disorders, left ventricular dysfunction, hypoxemia and cataract than the other DM1 patients.
CONCLUSION
The MFM provides an effective evaluation of muscular disability in DM1 patients. Cardiac and respiratory involvements are correlated with lower MFM.
背景
强直性肌营养不良症（DM1）是一种多系统疾病，其特征为强直性肌肉无力以及众多器官损害，尤其是心脏和呼吸系统疾病。本研究的目的是评估在DM1患者中一种新的肌肉残疾量表——运动功能测量（MFM）与器官受累功能测量之间的关系。
患者与方法
对69例连续的DM1患者进行了MFM和改良肌营养不良评定量表（MDRS）评估，以及肺活量测定、血气分析、超声心动图、心电图和眼科检查。
结果
发现MDRS与MFM之间存在显著相关性（p < 0.001），且二者与患者年龄和体重指数也存在显著相关性。MFM总分低于76的患者比其他DM1患者发生呼吸功能不全、传导障碍、左心室功能障碍、低氧血症和白内障的风险更高。
结论
MFM能有效评估DM1患者的肌肉残疾情况。心脏和呼吸系统受累与较低的MFM相关。
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