Physiology-Based Treatment of Myoclonus

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.