肌强直

Patients have paradoxic myotonia (myotonia that worsens with repeated activity) and periodic paralysis.

患者表现为反常性肌强直（即在活动中出现肌强直，随持续或反复活动而加重）和周期性麻痹。

Results There were total 68 people in 5 generations, including 24 patients in 4 generations .Both male and female were suffered. All patientes of this kindred showed myotonia with onset the illness from the infant, and 16 cases accompanied with hypermyotrophy.

结果 该家系5代68名成员，连续4代共24例发病，男女均有累及；多于婴幼儿期起病，肌强直见于所有患者，16例伴有肌肥大。

Clinical features include bone abnormalities, dwarfism, facial and ocular anomalies, gait difficulties, and severe voluntary and percussion myotonia.

临床特点包括骨组织异常、侏儒症、面部及眼部异常、步行困难、严重的随意运动性肌强直和叩击性肌强直。

Myotonic dystrophy is usually obvious on clinical grounds with grip myotonia, distal weakness and wasting and the characteristic facial appearance, and was not considered likely here.

在临床见到的明显的营养不良性肌强直常表现为紧握的肌强直，远端无力和萎缩，以及特征性面容。因而在这位患者并不考虑本病。

These conditions are distinguished by the presence of clinical and electrical myotonia and a delay in muscle relaxation after contraction because of prolonged excitation of the muscle membrane.

肌强直综合征可以通过以下表现加以鉴别：临床检查及电生理检测军发现肌强直、因肌膜兴奋性延长所致的肌肉收缩后松弛延时缓慢。

Another feature of this syndrome is delayed muscle relaxation, which differs from true myotonia by absent percussion myotonia and increased rather than decreased stiffness during continued activity.

Isaacs综合征的另一个特点是肌肉松弛延缓。1。 Isaacs综合征患者，无叩击性肌强直；2。持续活动期间，肌强直加剧而非减轻。

Current neurological examination disclosed percussion myotonia and myotonia as and DNA analysis. The patient's mother, though minimally affected, had similar laboratory results.

神经学检查，发现有肌强直现象，并由典型的肌电图变化及脱氧核醣核酸分析，确定为先天型肌强直失养症的此病。

DM1 and DM2 gene were amplified by PCR,tested by agarose electrophoresis, then analyzed by genetic analyzer. Results: Myotonia and muscle weakness were the main manifestations associated with heart block (7/8) and cataract (6/7). Electromyologram showed myopathic abnormalities not only in patients but also in other members of the family (5/6). The CTG repeats in DM1 and CCTG repeats in DM2 were all in normal range.

结果：该家系患者除有肌强直、肌萎缩等表现外；心电图检查：心脏传导阻滞(7/8)；裂隙灯检查：白内障(6/7)；肌电图检查：患者组有强直电位发放(5/5)，无临床症状成员也存在肌源性损害(5/6)；但该家系无DM1位点n和DM2位点n的重复数增加。

The decoction relieved muscle rigidity more effectively than tremor.It could improve hypokinesia and quality of life fundamentally.

补肾活血饮对强直明显者疗效较震颤者显著，说明其能明显缓解肌强直，从根本上改善患者的运动功能和日常生活能力。

Quick movements cause muscle stiffen ing. There is also a myotonic form of muscular dystrophy.

快速运动会引起肌强直，肌营养不良是另一种肌强直类型。

According to the clear water experiment, aeration performance of the new equipment is good with high total oxygen transfer coefficient and oxygen utilization ratio.

曝气设备的动力效率在叶轮转速为120rpm～150rpm时取得最大值，此时氧利用率和充氧能力也具有较高值。

The environmental stability of that world - including its crushing pressures and icy darkness - means that some of its most famous inhabitants have survived for eons as evolutionary throwbacks, their bodies undergoing little change.

稳定的海底环境─包括能把人压扁的压力和冰冷的黑暗─意谓海底某些最知名的栖居生物已以演化返祖的样态活了万世，形体几无变化。