Physiology-Based Treatment of Myoclonus
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Physiology-Based Treatment of Myoclonus Ashley B. Pena 1
&
John N. Caviness 2
# The American Society for Experimental NeuroTherapeutics, Inc. 2020
Abstract Myoclonus can cause significant disability for patients. Myoclonus has a strikingly diverse array of underlying etiologies, clinical presentations, and pathophysiological mechanisms. Treatment of myoclonus is vital to improving the quality of life of patients with these disorders. The optimal treatment strategy for myoclonus is best determined based upon careful evaluation and consideration of the underlying etiology and neurophysiological classification. Electrophysiological testing including EEG (electroencephalogram) and EMG (electromyogram) data is helpful in determining the neurophysiological classification of myoclonus. The neurophysiological subtypes of myoclonus include cortical, cortical–subcortical, subcortical–nonsegmental, segmental, and peripheral. Levetiracetam, valproic acid, and clonazepam are often used to treat cortical myoclonus. In cortical–subcortical myoclonus, treatment of myoclonic seizures is prioritized, valproic acid being the mainstay of therapy. Subcortical–nonsegmental myoclonus may be treated with clonazepam, though numerous agents have been used depending on the etiology. Segmental and peripheral myoclonus are often resistant to treatment, but anticonvulsants and botulinum toxin injections may be of utility depending upon the case. Pharmacological treatments are often hampered by scarce evidence-based knowledge, adverse effects, and variable efficacy of medications. Keywords Myoclonus . treatment . EMG . EEG . neurophysiology
Introduction Myoclonus is defined as sudden, brief, lightning-like involuntary muscle jerks arising from the nervous system. Myoclonus may result from abrupt abnormal muscle activation or inhibition of muscle activation (negative myoclonus) [1]. Myoclonus can occur at rest, in response to stimulation, upon muscle activation, or any combination thereof. Such
Electronic supplementary material The online version of this article (https://doi.org/10.1007/s13311-020-00922-6) contains supplementary material, which is available to authorized users. * Ashley B. Pena [email protected] John N. Caviness [email protected] 1
Department of Neurology, Mayo Clinic Florida, 4500 San Pablo Rd S, Jacksonville, Florida 32224, USA
2
Department of Neurology, Mayo Clinic Arizona, 13400 East Shea Blvd., Scottsdale, Arizona 85259, USA
intrusions into normal movement disrupt activities of daily living, causing significant disability [2]. Myoclonus may occur in generalized, focal, multifocal, or segmental distributions. More than one distribution could be seen in the same patient or disease process. Identification of myoclonus can be challenging as other conditions cause brief jerking movements that may resemble myoclonus clinically. Such conditions could include dystonia, chorea, ballism, motor tics, and functional jerks [3]. Further, myoclonus may be subtle and can occur in repetitive fashion causing it to resemb
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