Jamieson P W, Katirji M B
Department of Neurology, University of Kansas Medical Center, Kansas City 66160-7314.
Muscle Nerve. 1994 Jan;17(1):42-51. doi: 10.1002/mus.880170106.
Idiopathic generalized myokymia (IGM) is a rare, heterogeneous, and poorly understood syndrome. We present analysis of 75 reported cases in the world literature. IGM affects men and women equally, with a mean age of onset 29 +/- 19 years. Patients' common presenting complaints are stiffness (60%), cramps (12%), weakness (12%), and muscle twitching (4%). Family history is positive in 30%. In addition to generalized clinical myokymia (92%), abnormal neurologic findings include: hyporeflexia (70%), weakness (45%), grip myotonia (39%), and calf hypertrophy (16%). Electrical activity consisting of spontaneous continuous motor unit activity and/or electrical myokymia was documented in all patients. When electrical myokymia was observed (66%), the grouped discharges where irregular and had an interburst frequency of 2-300 Hz. Both phenytoin and carbamazepine are effective treatments. We conclude that IGM has a wide spectrum of symptoms and severity and should be considered in all patients that present with stiffness, cramps, or muscle twitching. EMG greatly aids in diagnosis.
特发性全身性肌束震颤综合征（IGM）是一种罕见、异质性且了解甚少的综合征。我们对世界文献中报道的75例病例进行了分析。IGM在男性和女性中发病率相同，平均发病年龄为29±19岁。患者常见的主诉症状有僵硬（60%）、痉挛（12%）、无力（12%）和肌肉抽搐（4%）。30%的患者有家族史阳性。除了全身性临床肌束震颤（92%）外，异常神经系统表现还包括：反射减退（70%）、无力（45%）、抓握性肌强直（39%）和小腿肥大（16%）。所有患者均记录到由自发持续性运动单位活动和/或肌电肌束震颤组成的电活动。当观察到肌电肌束震颤时（66%），成组放电不规则，爆发间期频率为2 - 300赫兹。苯妥英和卡马西平均为有效的治疗方法。我们得出结论，IGM有广泛的症状和严重程度范围，所有出现僵硬、痉挛或肌肉抽搐的患者都应考虑该病。肌电图对诊断有很大帮助。
