Abstract
Few movement disorders seem to make a straightforward approach to diagnosis and treatment more difficult and frustrating than myoclonus, due to its plethora of causes and its variable classifications. Nevertheless, in recent years, exciting advances have been made in the elucidation of the pathophysiology and genetic basis of many disorders presenting with myoclonus. Here, we provide a review of all of the important types of myoclonus encountered in pediatric and adult neurology, with an emphasis on the recent developments that have led to a deeper understanding of this intriguing phenomenon. An up-to-date list of the genetic basis of all major myoclonic disorders is presented. Randomized studies are scarce in myoclonus therapy, but helpful pragmatic approaches at diagnosis as well as treatment have been recently suggested.
Highlights
Myoclonus is characterized by sudden, brief, shock-like involuntary movements, associated with bursts of muscular activity or silencing of muscular activity [1]
Following the subdivision of pyramidal, extrapyramidal and segmental myoclonus suggested by Halliday (1967) [8], the anatomical categorization has been refined in recent years based on electrodiagnostic studies
Cortical myoclonus is a condition typically characterized by abnormally enlarged somatosensory evoked potentials, exaggerated long latency reflexes (LLR)
Summary
Myoclonus is characterized by sudden, brief, shock-like involuntary movements, associated with bursts of muscular activity (positive myoclonus) or silencing of muscular activity (negative myoclonus) [1] It may be present at rest, during voluntary movement (action-induced) or due to provoking stimuli such as sensory, visual, auditory or emotional cues. Myoclonus presents as short (10–50 ms, rarely more than 100 ms), non-rhythmic jerks, often without any discernible pattern. Some cases of cortical tremors have been identified as cortical myoclonus based on electrophysiology, for example [3]. In these instances, agonist and antagonist muscles are involved simultaneously; a feature that is rather rare in tremors. Etiology focal segmental axial multifocal generalized rest action stimulus-induced (sensory, visual, auditory, emotional) cortical cortical-subcortical subcortical segmental (peripheral) physiologic essential epileptic symptomatic functional
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
