{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,24]],"date-time":"2026-08-24T09:21:07Z","timestamp":1787563267675,"version":"build-2736575974"},"reference-count":121,"publisher":"Oxford University Press (OUP)","issue":"9","license":[{"start":{"date-parts":[[2020,5,17]],"date-time":"2020-05-17T00:00:00Z","timestamp":1589673600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"name":"Horizon 2020 EU","award":["634821"],"award-info":[{"award-number":["634821"]}]},{"name":"National Institute for Health Research University College London Hospitals Biomedical Research Centre"},{"DOI":"10.13039\/501100003136","name":"Edmond J. Safra Philanthropic Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003136","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020,9,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Cortical tremor is a fine rhythmic oscillation involving distal upper limbs, linked to increased sensorimotor cortex excitability, as seen in cortical myoclonus. Cortical tremor is the hallmark feature of autosomal dominant familial cortical myoclonic tremor and epilepsy (FCMTE), a syndrome not yet officially recognized and characterized by clinical and genetic heterogeneity. Non-coding repeat expansions in different genes have been recently recognized to play an essential role in its pathogenesis. Cortical tremor is considered a rhythmic variant of cortical myoclonus and is part of the \u2018spectrum of cortical myoclonus\u2019, i.e. a wide range of clinical motor phenomena, from reflex myoclonus to myoclonic epilepsy, caused by abnormal sensorimotor cortical discharges. The aim of this update is to provide a detailed analysis of the mechanisms defining cortical tremor, as seen in FCMTE. After reviewing the clinical and genetic features of FCMTE, we discuss the possible mechanisms generating the distinct elements of the cortical myoclonus spectrum, and how cortical tremor fits into it. We propose that the spectrum is due to the evolution from a spatially limited focus of excitability to recruitment of more complex mechanisms capable of sustaining repetitive activity, overcoming inhibitory mechanisms that restrict excitatory bursts, and engaging wide areas of cortex. Finally, we provide evidence for a possible common denominator of the elements of the spectrum, i.e. the cerebellum, and discuss its role in FCMTE, according to recent genetic findings.<\/jats:p>","DOI":"10.1093\/brain\/awaa129","type":"journal-article","created":{"date-parts":[[2020,4,11]],"date-time":"2020-04-11T07:10:32Z","timestamp":1586589032000},"page":"2653-2663","source":"Crossref","is-referenced-by-count":61,"title":["Unravelling the enigma of cortical tremor and other forms of cortical myoclonus"],"prefix":"10.1093","volume":"143","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7103-5209","authenticated-orcid":false,"given":"Anna","family":"Latorre","sequence":"first","affiliation":[{"name":"Department of Clinical and Movement Neurosciences, Queen Square Institute of Neurology, University College London, London, UK"},{"name":"Department of Human Neurosciences, Sapienza University of Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lorenzo","family":"Rocchi","sequence":"additional","affiliation":[{"name":"Department of Clinical and Movement Neurosciences, Queen Square Institute of Neurology, University College London, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4706-6245","authenticated-orcid":false,"given":"Francesca","family":"Magrinelli","sequence":"additional","affiliation":[{"name":"Department of Clinical and Movement Neurosciences, Queen Square Institute of Neurology, University College London, London, UK"},{"name":"Department of Neurosciences, Biomedicine and Movement Sciences, University of Verona, Verona, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eoin","family":"Mulroy","sequence":"additional","affiliation":[{"name":"Department of Clinical and Movement Neurosciences, Queen Square Institute of Neurology, University College London, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alfredo","family":"Berardelli","sequence":"additional","affiliation":[{"name":"Department of Human Neurosciences, Sapienza University of Rome, Italy"},{"name":"Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Neuromed, Pozzilli, IS, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John C","family":"Rothwell","sequence":"additional","affiliation":[{"name":"Department of Clinical and Movement Neurosciences, Queen Square Institute of Neurology, University College London, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kailash P","family":"Bhatia","sequence":"additional","affiliation":[{"name":"Department of Clinical and Movement Neurosciences, Queen Square Institute of Neurology, University College London, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2020,5,17]]},"reference":[{"key":"2020111212324555300_awaa129-B1","doi-asserted-by":"crossref","first-page":"F3","DOI":"10.1186\/1471-2202-15-S1-F3","article-title":"Inhibitory single neuron control of seizures and epileptic traveling waves in humans","volume":"15","author":"Ahmed","year":"2014","journal-title":"BMC Neurosci"},{"key":"2020111212324555300_awaa129-B2","doi-asserted-by":"crossref","first-page":"1024","DOI":"10.1002\/mds.27331","article-title":"Cerebellar and brainstem functional abnormalities in patients with primary orthostatic tremor","volume":"33","author":"Antelmi","year":"2018","journal-title":"Mov Disord"},{"key":"2020111212324555300_awaa129-B3","doi-asserted-by":"crossref","first-page":"1087","DOI":"10.1093\/brain\/118.5.1087","article-title":"Progressive myoclonic ataxia associated with coeliac disease. 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