Myoclonus: Differential diagnosis and current management

Author:

Riva Antonella12ORCID,D'Onofrio Gianluca12ORCID,Ferlazzo Edoardo34,Pascarella Angelo34ORCID,Pasini Elena5ORCID,Franceschetti Silvana6,Panzica Ferruccio6,Canafoglia Laura6ORCID,Vignoli Aglaia7,Coppola Antonietta8ORCID,Badioni Valeria9,Beccaria Francesca10,Labate Angelo11ORCID,Gambardella Antonio3ORCID,Romeo Antonino12ORCID,Capovilla Giuseppe13,Michelucci Roberto5,Striano Pasquale12ORCID,Belcastro Vincenzo9

Affiliation:

1. Department of Neurosciences Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI) University of Genoa Genoa Italy

2. Pediatric Neurology and Muscular Diseases Unit IRCCS Istituto “Giannina Gaslini” Genoa Italy

3. Department of Medical and Surgical Sciences Magna Græcia University of Catanzaro Catanzaro Italy

4. Regional Epilepsy Centre Great Metropolitan “Bianchi‐Melacrino‐Morelli Hospital” Reggio Calabria Italy

5. IRCCS‐Istituto delle Scienze Neurologiche di Bologna, Unit of Neurology Bellaria Hospital Bologna Italy

6. Department of Diagnostics and Technology Fondazione IRCCS Istituto Neurologio Carlo Besta Milan Italy

7. Childhood and Adolescence Neurology and Psychiatry Unit, ASST GOM Niguarda, Health Sciences Department Università degli Studi di Milano Milano Italy

8. Department of Neuroscience, Odontostomatology and Reproductive Sciences Federico II University of Naples Naples Italy

9. Neurology Unit ASST Lodi Lodi Italy

10. ASST di Lodi Lodi Italy

11. Neurophysiology and Movement Disorders Unit University of Messina Messina Italy

12. Pediatric Neurology Unit and Epilepsy Center, Department of Neuroscience “Fatebenefratelli e Oftalmico" Hospital Milano Italy

13. Fondazione Poliambulanza Brescia Italy

Abstract

AbstractMyoclonus classically presents as a brief (10–50 ms duration), non‐rhythmic jerk movement. The etiology could vary considerably ranging from self‐limited to chronic or even progressive disorders, the latter falling into encephalopathic pictures that need a prompt diagnosis. Beyond the etiological classification, others evaluate myoclonus' body distribution (i.e., clinical classification) or the location of the generator (i.e., neurophysiological classification); particularly, knowing the anatomical source of myoclonus gives inputs on the observable clinical patterns, such as EMG bursts duration or EEG correlate, and guides the therapeutic choices. Among all the chronic disorders, myoclonus often presents itself as a manifestation of epilepsy. In this context, myoclonus has many facets. Myoclonus occurs as one, or the only, seizure manifestation while it can also present as a peculiar type of movement disorder; moreover, its electroclinical features within specific genetically determined epileptic syndromes have seldom been investigated. In this review, following a meeting of recognized experts, we provide an up‐to‐date overview of the neurophysiology and nosology surrounding myoclonus. Through the dedicated exploration of epileptic syndromes, coupled with pragmatic guidance, we aim to furnish clinicians and researchers alike with practical advice for heightened diagnostic management and refined treatment strategies.Plain Language SummaryIn this work, we described myoclonus, a movement characterized by brief, shock‐like jerks. Myoclonus could be present in different diseases and its correct diagnosis helps treatment.

Publisher

Wiley

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