Myokymemia Notes

Adams & Victors

Myokymia: simultaneous / sequential spontaneous contractions of multiple motor units causing a rippling of muscle (39)
o Multiple motor units involved, each firing at its own rate, quasi-rhythmically (1104)
o Most commonly seen with regeneration of peripheral nerve following injuries (1278)
Persistent quivering and rippling of muscles at rest (live flesh) (1104)
Always has peripheral activation, although disputed site of origin
Causes of myokymia
o Demyleniating polyneuropathies
o Peripheral nerve hyperexcitability due to K+ channel mutations and antibodies against channels
Generalized vs. segmental vs. focal myokymia
o Isaacs syndrome (page 1278): generalized myokymia
EMG discloses high-frequency (up to 300 Hz) repititve discharges of varying waveforms
o Focal = mainly facial myokymi (MS), GBS, large cerebellopontine angle tumors, compression of facial nerve
o Segmental myokymia: common in radiation injuries of brachial plexus
EMG bursts longer and less frequent than in generalized
Interfrequency bursts more variable
AKA neuromyotonia
Occurs during sleep and general anesthesia
Cf. benign fasciculations: will havenormal EMGs
o A simple clinical rule is that fasciculations in relaxed muscle are not indicative of motor system disease unless
there is weakness, atrophy, or reflex change (1277)
Clinical features
o The tendon reflexes may be reduced or abolished. Any muscle group may be affected. The stiffness and slowness of
movement make walking laborious (armadillo syndrome); in extreme cases, all voluntary movement is blocked.
The muscle activity persists throughout sleep. (1278)