Myoclonus and other jerky movement disorders

Sterre van der Veen; John N Caviness; Yasmine E M Dreissen; Christos Ganos; Abubaker Ibrahim; Johannes H T M Koelman; Ambra Stefani; Marina A J Tijssen

doi:10.1016/j.cnp.2022.09.003

Myoclonus and other jerky movement disorders

Clin Neurophysiol Pract. 2022 Oct 6:7:285-316. doi: 10.1016/j.cnp.2022.09.003. eCollection 2022.

Authors

Sterre van der Veen^{1

2}, John N Caviness³, Yasmine E M Dreissen⁴, Christos Ganos⁵, Abubaker Ibrahim⁶, Johannes H T M Koelman⁷, Ambra Stefani⁶, Marina A J Tijssen^{1

2}

Affiliations

¹ Department of Neurology, University of Groningen, University Medical Centre Groningen (UMCG), Groningen, The Netherlands.
² Expertise Centre Movement Disorders Groningen, University Medical Centre Groningen (UMCG), Groningen, The Netherlands.
³ Department of Neurology, Mayo Clinic Arizona, Movement Neurophysiology Laboratory, Scottsdale, AZ, USA.
⁴ Department of Neurosurgery, Amsterdam University Medical Centers, Amsterdam, The Netherlands.
⁵ Movement Disorders and Neuromodulation Unit, Department of Neurology, Charité University Medicine Berlin, Berlin, Germany.
⁶ Department of Neurology, Medical University of Innsbruck, Innsbruck, Austria.
⁷ Department of Neurology and Clinical Neurophysiology, Amsterdam University Medical Centers, Amsterdam, The Netherlands.

Abstract

Myoclonus and other jerky movements form a large heterogeneous group of disorders. Clinical neurophysiology studies can have an important contribution to support diagnosis but also to gain insight in the pathophysiology of different kind of jerks. This review focuses on myoclonus, tics, startle disorders, restless legs syndrome, and periodic leg movements during sleep. Myoclonus is defined as brief, shock-like movements, and subtypes can be classified based the anatomical origin. Both the clinical phenotype and the neurophysiological tests support this classification: cortical, cortical-subcortical, subcortical/non-segmental, segmental, peripheral, and functional jerks. The most important techniques used are polymyography and the combination of electromyography-electroencephalography focused on jerk-locked back-averaging, cortico-muscular coherence, and the Bereitschaftspotential. Clinically, the differential diagnosis of myoclonus includes tics, and this diagnosis is mainly based on the history with premonitory urges and the ability to suppress the tic. Electrophysiological tests are mainly applied in a research setting and include the Bereitschaftspotential, local field potentials, transcranial magnetic stimulation, and pre-pulse inhibition. Jerks due to a startling stimulus form the group of startle syndromes. This group includes disorders with an exaggerated startle reflex, such as hyperekplexia and stiff person syndrome, but also neuropsychiatric and stimulus-induced disorders. For these disorders polymyography combined with a startling stimulus can be useful to determine the pattern of muscle activation and thus the diagnosis. Assessment of symptoms in restless legs syndrome and periodic leg movements during sleep can be performed with different validated scoring criteria with the help of electromyography.

Keywords: Deep Brain Stimulation; EEG; EMG; Local field potentials; Myoclonus; Neurophysiology; PLMS; RLS; Startle; Tics; Tourette disorder; Transcranial Magnetic Stimulation.

Publication types

Review