John N. Caviness, MD, FAAN p. 1055-1080 August 2019, Vol.25, No.4 doi: 10.1212/CON.0000000000000750
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PURPOSE OF REVIEW: This article offers clinicians a strategic approach for making sense of a symptom complex that contains myoclonus. The article presents an evaluation strategy that highly leverages the two major classification schemes of myoclonus. The goal of this article is to link evaluation strategy with diagnosis and treatment of myoclonus.

RECENT FINDINGS: The growth of medical literature has helped better define myoclonus etiologies. Physiologic study of myoclonus types and etiologies with electrophysiologic testing has provided greater clarity to the pathophysiology of the myoclonus in various diseases. Although studies have been limited, the role of newer treatment agents and methods has made progress.

SUMMARY: Myoclonus has hundreds of different etiologies. Classification is necessary to evaluate myoclonus efficiently and pragmatically. The classification of myoclonus etiology, which is grouped by different clinical presentations, helps determine the etiology and treatment of the myoclonus. The classification of myoclonus physiology using electrophysiologic test results helps determine the pathophysiology of the myoclonus and can be used to strategize symptomatic treatment approaches. Both basic ancillary testing (including EEG and imaging) and more comprehensive testing may be necessary. Treatment of the underlying etiology is the ideal approach. However, if such treatment is not possible or is delayed, symptomatic treatment guided by the myoclonus physiology should be considered. More controlled study of myoclonus treatment is needed. Further research on myoclonus generation mechanisms should shed light on future treatment possibilities.

Address correspondence to Dr John N. Caviness, Department of Neurology, Mayo Clinic 13400 E Shea Blvd, Scottsdale, AZ 85259,

RELATIONSHIP DISCLOSURE: Dr Caviness reports no disclosure.

UNLABELED USE OF PRODUCTS/INVESTIGATIONAL USE DISCLOSURE: Dr Caviness discusses the unlabeled/investigational use of anticholinergic medications, botulinum toxin, carbamazepine, clonazepam, deep brain stimulation, levetiracetam, sodium oxybate, and tetrabenazine for the treatment of myoclonus.

© 2019 American Academy of Neurology.