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.