Haass A, Ricker K, Rüdel R, Lehmann-Horn F, Böhlen R, Dengler R, Mertens H G
Muscle Nerve. 1981 Sep-Oct;4(5):388-95. doi: 10.1002/mus.880040507.
Fourteen patients with paramyotonia congenita were examined clinically. Patients of 3 families had no myotonia in a warm environment while in a cold environment they developed paradoxical myotonia (myotonia aggravated by repeated muscle contraction). Patients of a 4th family had myotonia associated with after-activity in a warm environment which was not paradoxical. This myotonia was aggravated by cooling. In a warm environment the resting muscles of all patients showed no spontaneous electromyographic activity except for occasional myotonic runs. On cooling, spontaneous fibrillations developed. This was most intense at 32 degrees C-28 degrees C (muscle temperature). On deeper cooling it ceased. In contrast, 5 patients with myotonia congenita did not show such activity during cooling. In all paramyotonic patients cooling (30 degrees C-25 degrees C) produced muscle paralysis, which outlasted rewarming by several hours. At 32 degrees C-30 degrees C muscle relaxation was slowed. Recording of electromyographic activity and isometric contractions of the long finger flexors during cooling revealed that the slowing of muscle relaxation in paramyotonia is not as closely linked to after-activity as is the slowing of muscle relaxation in myotonia congenita.
对14例先天性副肌强直患者进行了临床检查。3个家族的患者在温暖环境中无肌强直，而在寒冷环境中会出现反常性肌强直（反复肌肉收缩会加重肌强直）。第4个家族的患者在温暖环境中活动后出现非反常性肌强直，这种肌强直在降温时会加重。在温暖环境中，所有患者的静息肌肉除偶尔出现肌强直放电外，无自发肌电图活动。降温时会出现自发纤颤，在32摄氏度至28摄氏度（肌肉温度）时最为强烈，进一步降温时则停止。相比之下，5例先天性肌强直患者在降温过程中未出现此类活动。在所有副肌强直患者中，降温（30摄氏度至25摄氏度）会导致肌肉麻痹，且在复温后仍持续数小时。在32摄氏度至30摄氏度时，肌肉松弛减慢。在降温过程中记录长指屈肌的肌电图活动和等长收缩发现，副肌强直中肌肉松弛减慢与活动后的关系不如先天性肌强直中肌肉松弛减慢与活动后的关系紧密。
