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Myokymia, a form of involuntary muscular movement, usually can be visualized on the skin as vermicular or continuous rippling movements.

The word myokymia was used first more than 100 years ago, when Schultze described continuous, slow, undulating muscular contractions in small muscles of hands and feet. [1] Kny used the term myoclonus fibrillaris multiplex to describe similar clinical manifestations. [2] For the past century, different authors applied the term myokymia to different involuntary muscular movements. Most of them showed electromyographic (EMG) evidence of spontaneous group discharges. This led to tremendous confusion in conceptually defining this particular clinical entity and its electrophysiologic features.

The clinical phenomenon is characterized by its classic quivering movement of the involved muscle without movement of the joint. Myokymia can be seen in muscles innervated by cranial or spinal nerves. The distribution can be either regional or generalized, depending on the etiology. Also, it can be seen transiently in healthy subjects after strenuous exercise.

The exact mechanism(s) of myokymia is not well understood. Myokymia of the facial muscles is believed to originate from the facial nucleus or from some contribution by a supranucleus process; however, the presence of myokymia in polyradiculopathy indicates the possibility of a more distal generator. Most authors agree that myokymia in other parts of the body is generated by distal motor axons, either by a primarily axonal process or by segmental demyelination with secondary axonal dysfunction. Some have postulated that transaxonal ephaptic excitation occurs peripherally after focal nerve damage leads to formation of an artificial synapse.

Myokymia is believed to be associated with generation of spontaneous activity, including myokymialike discharge in the dystrophic mouse whose nerve root axons have no Schwann-cell enwrapment. By this mechanism, spontaneous discharge could initiate volleys of activity or afferent fibers could directly stimulate efferent fibers in the vicinity of the lesion and produce a self-perpetuating reverberating circuit.

The central nervous system’s electrotonic spread of discharge from rhythmic generators toward anterior horn cells also might play a role in generation of the spontaneous discharge. Each patient may have a different operating mechanism, depending on the particular areas involved and the different etiologies. The fact that patients with Isaacs syndrome respond dramatically to treatment of myokymia with phenytoin and/or carbamazepine [3] suggests a possible abnormality of the potassium channel in this particular entity.

Although myokymia can be seen in patients with different neurological and medical conditions and occasionally even in healthy subjects, it is a relatively rare clinical manifestation.

Most of the diseases associated with myokymia are not life threatening.

The prognosis is solely dependent upon the underlying etiologies.

Myokymia is considered benign when detected in patients after strenuous exercise.

Prognosis is related directly to the underlying etiology. Myokymia is reversible with successful treatment of the cause. [9]

Schultze F. Beitrage zur Muskelpathologie. Deutsch Z Nervenheilk. 1895. 6:65-167.

Kny E. Ueber ein dem Paramyoclonus multiplex (Friedreich) nahestehendes Krankheitsbild. Arch Psychiat Nervenkr. 1888. 19:577.

Jackson DL, Satya-Murti S, Davis L, Drachman DB. Isaacs syndrome with laryngeal involvement: an unusual presentation of myokymia. Neurology. 1979 Dec. 29(12):1612-5. [Medline].

Isaacs H. A syndrome of continuous muscle-fibre activity. J Neurol Neurosurg Psychiat. 1961. 24:319-325.

Doi H, Arimura K, Ohyagi Y, Kira J. Frostbite-like skin lesion as an autonomic symptom of Isaacs’ syndrome. Intern Med. 2011. 50(10):1113-5. [Medline].

Hayashi Y, Kimura A, Watanabe N, Yamada M, Sakurai T, Tanaka Y, et al. Serial monitoring of basal metabolic rate for therapeutic evaluation in an Isaacs’ syndrome patient with chronic fluctuating symptoms. Intern Med. 2010. 49(5):475-7. [Medline].

Lide B, Singh J, Haeri S. Isaacs’ syndrome in pregnancy. BMJ Case Rep. 2014 Oct 9. 2014:[Medline].

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Moghimi N, Rosen JB, Jabbari B. Ten Years’ Follow-Up of a Family With Myokymia and Muscle Cramps Without Ataxia. J Child Neurol. 2013 Nov. 28(11):1493-1495. [Medline].

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Chinskey ND, Cornblath WT. Inferior oblique myokymia: a unique ocular motility disorder. JAMA Ophthalmol. 2013 Mar. 131(3):404-5. [Medline].

Denny-Brown D, Foley JM. Myokymia and the benign fasciculation of muscular cramps. Trans Assoc Am Physicians. 1948. 61:88-96.

Fernandez M, Raskind W, Wolff J, Matsushita M, Yuen E, Graf W. Familial dyskinesia and facial myokymia (FDFM): a novel movement disorder. Ann Neurol. 2001 Apr. 49(4):486-92. [Medline].

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Greenhouse AH, Bicknell JM, Pesch RN, Seelinger DF. Myotonia, myokymia, hyperhidrois and wasting of muscle. Neurology. 1967 Mar. 17(3):263-8. [Medline].

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Isaacs H, Frere G. Syndrome of continuous muscle fibre activity. Histochemical, nerve terminal and end-plate study of two cases. S Afr Med J. 1974 Aug 10. 48(38):1601-7. [Medline].

Lance JW, Burke D, Pollard J. Hyperexcitability of motor and sensory neurons in neuromyotonia. Ann Neurol. 1979 Jun. 5(6):523-32. [Medline].

Lublin FD, Tsairis P, Streletz LJ, et al. Myokymia and impaired muscular relaxation with continuous motor unit activity. J Neurol Neurosurg Psychiatry. 1979 Jun. 42(6):557-62. [Medline].

Lutschg J, Jerusalem F, Ludin HP, et al. The syndrome of ‘continuous muscle fiber activity.’. Arch Neurol. 1978 Apr. 35(4):198-205. [Medline].

Matthews WB. Facial myokymia. J Neurol Neurosurg Psychiatry. 1966 Feb. 29(1):35-9. [Medline].

Medina JL, Chokroverty S, Reyes M. Localized myokymia caused by peripheral nerve injury. Arch Neurol. 1976 Aug. 33(8):587-8. [Medline].

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Solimena M, Folli F, Denis-Donini S, et al. Autoantibodies to glutamic acid decarboxylase in a patient with stiff-man syndrome, epilepsy, and type I diabetes mellitus. N Engl J Med. 1988 Apr 21. 318(16):1012-20. [Medline].

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Wallis WE, Van Poznak A, Plum F. Generalized muscular stiffness, fasciculations, and myokymia of peripheral nerve origin. Arch Neurol. 1970 May. 22(5):430-9. [Medline].

Welch LK, Appenzeller O, Bicknell JM. Peripheral neuropathy with myokymia, sustained muscular contraction, and continuous motor unit activity. Neurology. 1972 Feb. 22(2):161-9. [Medline].

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Suying L Song, MD Assistant Clinical Professor of Neurology, New York University School of Medicine

Suying L Song, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Neil A Busis, MD Chief of Neurology and Director of Neurodiagnostic Laboratory, UPMC Shadyside; Clinical Professor of Neurology and Director of Community Neurology, Department of Neurology, University of Pittsburgh Physicians

Neil A Busis, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: American Academy of Neurology<br/>Serve(d) as a speaker or a member of a speakers bureau for: American Academy of Neurology<br/>Received income in an amount equal to or greater than $250 from: American Academy of Neurology.

Nicholas Lorenzo, MD, MHA, CPE Co-Founder and Former Chief Publishing Officer, eMedicine and eMedicine Health, Founding Editor-in-Chief, eMedicine Neurology; Founder and Former Chairman and CEO, Pearlsreview; Founder and CEO/CMO, PHLT Consultants; Chief Medical Officer, MeMD Inc

Nicholas Lorenzo, MD, MHA, CPE is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, American Association for Physician Leadership

Disclosure: Nothing to disclose.

Carmel Armon, MD, MSc, MHS Chair, Department of Neurology, Assaf Harofeh Medical Center, Tel Aviv University Sackler Faculty of Medicine, Israel

Carmel Armon, MD, MSc, MHS is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Association of Neuromuscular and Electrodiagnostic Medicine, American Clinical Neurophysiology Society, American College of Physicians, American Epilepsy Society, American Medical Association, American Neurological Association, American Stroke Association, Massachusetts Medical Society, Sigma Xi

Disclosure: Received research grant from: Neuronix Ltd, Yoqnea’m, Israel<br/>Received income in an amount equal to or greater than $250 from: JNS – Associate Editor. UpToDate – Author Royalties.


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