Facial Myokymia in the Guillain-Barre Syndrome

William R. Wasserstrom, MD, Arnold Starr, MD

• Facial myokymia, a unique involun­ kymia, indicating that these move­ contained one lymphocyte, no red blood tary movement of facial muscles, Is ments can also occur with lesions of cells, a protein level of 340 mg/100 ml, and described in a patient with Gulllain-Barre the facial nerve extrinsic to the brain a glucose level of 60 mg/ 100 ml. An electrfatigue, and myalgias. After distal weakness in all four extremities, kymia. five days, these symptoms subsided, but absent muscle stretch reflexes, and mild (Arch Neuro/ 34:576-577, 1977) were then followed in about three days by distal sensory loss to all modalities. One numbness and tingling in the distal aspects month later, involuntary facial movements of both hands and feet. One week later, she could not be detected. The reflexes were acial myokymia is a continuous, noted an involuntary twitching and present in the upper limbs, but still absent F involuntary, undulating move­ "worm-like" movement of the lower half of in lower limbs. The patient's strength was m~nt of the facial muscles. It has been the right side of her face. In the next few now intact, but the mild distal sensory described most prominently in asso­ days, these movements spread to involve deficits persisted. ciation with intramedullary brain the entire right side of the face and, to COMMENT stem lesions such as multiple sclero­ some extent, th~ lower portion of the left sis,' pontine gliomas, and other poste­ side of the face. On admission to the hospital in May of This case illustrates an infrequently rior fossa tumors;·• and occasionally observed clinical association of facial with poliomyelitis affecting the facial 1976, the examination revealed continuous flickering movements of the entire right myokymia with the Guillain-Barre nerve nucleus• and brain stem vascu­ side of the face extending from the syndrome.• Facial myokymia has been lar diseases.•·• Several cases of the platysma muscle to the frontalis. Similar well described in association with phenomenon have been described with movements of the buccal musculature of multiple sclerosis and intramedullary extramedullary brain stem tumors as the left side of the face were also evident. tumors of the pons, and rarely with acoustic scbwannoma,2 but in these There was no facial weakness or change in extramedullary tumors that compress instances, the tumors were of suffi­ facial sensation. Taste and hearing were the brain stem.,.. The facial myokym­ cient size to compress the brain stem. likewise intact. The patient had a mild ia in all of these cases has been Thus, facial myokymia has been degree of weakness in the lower extremi­ ties and in the distal musculature of the presumed to result froin abnormali­ considered a clinical sign of an intra­ ties within the brain stem affecting medullary brain stem lesion producing upper extremities, but fasciculations and myokymia were not present. All muscle the nucleus of the facial nerve. Tenser hyperexcitability (or perhaps loss of 3 stretch reflexes were absent, except for a and Corbett thought this hypothesis inhibitory control) in the nucleus of trace response from the right biceps. There was reasonable, since the myokymia the facial nerve. The patient described was mild symmetrical distal sensory was eliminated in a case of brain stem in this report had postinfectious poly­ impairment to all modalities in the extrem­ glioma by an anesthetic block of the radiculopathy (the Guillain-Barre syn­ ities. The remainder of the examination 7th cranial nerve at the stylomastoid drome) with prominent facial myo- was within normal limits. fora men. The involuntary facial movements di­ Facial myokymia is to be distin­ Accepted for publication April 25, 1977. minished, but did not totally disappear From the Department of Medicine (Neurolo­ during sleep. Analyses of the urine for guished from other involuntary facial gy), University of California, lrvine. porphobilinogen and heavy metals showed movements such as hemifacial spasm,' Reprint requests to Division of Neurology, University of California, Irvine, University oI no abnormalities. The cerebrospinal fluid facial contractures," facial synkinesias California Irvine Medical Center, 101 City Dr S, obtained from a lumbar puncture was of accompanying regeneration of the 7th Orange, CA 92668 (Dr Starr). normal pressure, but yellow-tinged. It cranial nerve, facial tics, tardive

576 Arch Neural-Vol 34, Sept 1977 Guiilain-Barre Syndrome-Wasserstrom & Starr dyskinesias, rhythmic facial contrac­ The persistence of myokymia, even stem and secondary changes in 7th­ tions occurring with palatal myoclo­ after spinal anesthesia, supports the nerve fiber could result from the brain nus, seizures from focal cortical idea that spontaneous activity origi­ stem lesion itself. However, it is more lesions, and fasciculations of facial nated from the peripheral nerves likely that facial myokymia is a neuro­ muscles with motor neuron disease. themselves, without participation of logical sign of increased excitability in These distinctions can be made both their cell bodies. Williamson and the facial motor system, beginning clinically and electrophysiologically. Brooke" agreed with the peripheral with the supranuclear pathways to the The characteristic EMG pattern of nerve or1gm of myokymia, and facial nucleus, the neurons of the facial myokymia is one of sponta­ referred to the work of Denny-Brown facial nucleus, the axons both within neous, rhythmic discharges of motor and Foley,13 who showed that repeated and extrinsic to the brain stem, and units appearing in singles, doubles, or bursts of action potentials character­ the neuromuscular junction at the groups, with relatively stable frequen­ istic of myokymia could occur in facial muscles. Lesions anywhere cy (between 0.8 and 30 cps). There normal individuals following the re­ along this pathway can result in the may be two types: continuous, in turn of blood flow to a nerve subjected characteristic involuntary movements which rhythmic single or double to ischemia for longer than 15 described as myokymia. discharges of one or a few motor units minutes. occur at a relatively high frequency, or In an unusual case described by discontinuous, in which rhythmic dis­ Welch et al," both facial and limb References charges of several units in groups muscle myokymia developed in a L Andermann F, Cosgrove JBR, Lloyd-Smith occur at a relatively lower frequen­ young woman. Though the authors do DL, et al: Facial myokymia in multiple sclerosis. cy.6 not specifically address themselves to Bra.in 84:31-44, 1961. In the patient presented in this the mechanisms of the facial move­ 2. Espinosa RE, Lambert EH, Klass OW: Facial myokymia affecting the electroencephalc>­ report, facial myokymia was the ments, they present evidence of gram. Ma.yo Clin Proc 42:258-270, 1967. result of a disease process causing segmental demyelination from a sural 3. Tenser RB, Corbett JJ: Myokymia and facial inflammation and demyelination of nerve biopsy to substantiate that a contraction in brainstem glioma. Arch Neurol 30:425-427, 1974. the facial nerve root• extrinsic to the peripheral nerve lesion was associated 4. Kaeser HE, Richter R, Wuthrich R: Les brain stem. It is possible that there with the myokymia. They postulated dyskinesies faciales. Rev Neurol 108:538-541, 1963. were accompanying retrograde the existence of increased excitability 5. Sethi PK, Smith BH, Kalyanaraman K: changes in the cells of the motor of the peripheral nerve as the mecha­ Facial myokymia: A clinicopathological study. J nucleus to provide depolarization and nism for the myokymia, since spinal Neural Neurosurg Psychiatry 37:74~749, 1974. 6. Radu EW, Skorpil V, Kaeser HE: Facial subsequent spontaneous discharges, anesthesia did not eliminate the myokymia. Eur Neurol· 13:499-512, 1975. but one might have expected some abnormal movement in their patient. 7. Eckman P, Kramer R, Altrocchi P: Hemifa­ It cial spasm. Arch Neurol 25:81-87, 1971. degree of facial weakness if this were seems plausible to consider that the 8. Sogg R, Hoyt W, Boldrey E: Spastic paretic true. facial nerve ·was also affected by the facial contracture. Neurology 13:607-612, 1963. Alternately, the peripheral nerve segmental demyelination. 9. Wisniewski H, Terry R, Whitaker JN, et al: Landry-Guillain-Barre syndrome. Arch Neurol root lesion itself may have resulted in Demyelination is a striking finding 21:269-276, 1969. the myokymia, in a manner similar to in patients with the Guillain-Barre 10. Medina J, Chokroverty S, Reyes M: Local­ the instances of myokymia known to syndrome, and the finding of facial ized myokymia caused by peripheral nerve 10 injury. Arch Neurol 33:587-588, 1976. occur with peripheral nerve lesions. myokymia may reflect the increased 11. Wallis W, Van Poznak A, Plum F: General­ Wallis et al" characterized myokymia spontaneous discharges of the facial ized muscular stiffness, fasciculation, and myo­ of peripheral nerve origin as being muscles as a consequence of this kymia of peripheral nerve origin. Arch Nwrol 22:43~439, 1970. multiple, continuous faciculations giv­ lesion. It may even be that the 12. Williamson E, Brooke M: Myokymia and ing the appearance of undulation myokymia seen in patients with the motor unit. Arch Neurol 26:11-16, 1972. IS. Denny-Brown D, Foley J: Myokymia and rather than isolated muscle twitches. multiple sclerosis or intramedullary the benign fasciculation of muscular cramps. They believed that the myokymia brain stem lesions can be caused by Trans Assoc Am Physicia.ns 61:88-96, 1948. represented spontaneous isolated dis­ the same peripheral nerve mech­ 14. Welch LK, Appenzeller 0, · Bicknell J: Peripheral neuropathy with myokymia, sus­ charges of individual motor units anisms, since the 7th cranial nerve has tained muscular contraction and continuous caused by the peripheral nerve lesion. a lengthy course through the brain motor unit activity. Neurolqgy 22:161-169, 1974.

Arch Neural-Vol 34, Sept 1977 Guillain-Barre Syndrome-Wasserstrom & Starr 577