Premium
Hereditary form of sustained muscle activity of peripheral nerve origin causing generalized myokymia and muscle stiffness
Author(s) -
Auger Raymond G.,
Daube Jasper R.,
Gomez Manuel R.,
Lambert Edward H.
Publication year - 1984
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410150104
Subject(s) - myokymia , motor unit , fasciculation , electromyography , motor nerve , peripheral , medicine , muscle biopsy , stimulus (psychology) , anatomy , motor neuron , anesthesia , biopsy , psychology , disease , physical medicine and rehabilitation , psychotherapist
Six patients in two unrelated families had a hereditary form of sustained muscle activity of peripheral nerve origin. Although varying in severity among affected family members, the disease was manifested clinically as generalized myokymia and muscle stiffness. Motor and sensory nerve conduction velocities and results of nerve biopsy were normal. Repetitive after‐discharges followed each motor nerve stimulus. Needle electrode examination demonstrated spontaneous recurrent bursts of motor unit activity. The burst activity of a motor unit occurred rhythmically (0.5 to 4 bursts per second) and independently of the activity of other units. Within a burst, the firing rate was high (150 to 300 Hz). The clinical and electromyographic abnormalities improved considerably after treatment with carbamazepine or phenytoin.