{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,24]],"date-time":"2026-06-24T23:54:43Z","timestamp":1782345283766,"version":"3.54.5"},"reference-count":44,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2018,8,19]],"date-time":"2018-08-19T00:00:00Z","timestamp":1534636800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2019,3]]},"abstract":"<jats:sec><jats:title>Background and purpose<\/jats:title><jats:p>Motor neuron disease (MND) refers to a spectrum of degenerative diseases affecting motor neurons. Recent clinical and post\u2010mortem observations have revealed considerable variability in the phenotype. Rhythmic involuntary oscillations of the hands during action, resembling tremor, can occur in MND, but their pathophysiology has not yet been investigated.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A total of 120 consecutive patients with MND were screened for tremor. Twelve patients with action tremor and no other movement disorders were found. Ten took part in the study. Tremor was recorded bilaterally using surface electromyography (EMG) and triaxial accelerometer, with and without a variable weight load. Power spectra of rectified EMG and accelerometric signal were calculated. To investigate a possible cerebellar involvement, eyeblink classic conditioning was performed in five patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Action tremor was present in about 10% of our population. All patients showed distal postural tremor of low amplitude and constant frequency, bilateral with a small degree of asymmetry. Two also showed simple kinetic tremor. A peak at the EMG and accelerometric recordings ranging from 4 to 12 Hz was found in all patients. Loading did not change peak frequency in either the electromyographic or accelerometric power spectra. Compared with healthy volunteers, patients had a smaller number of conditioned responses during eyeblink classic conditioning.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Our data suggest that patients with MND can present with action tremor of a central origin, possibly due to a cerebellar dysfunction. This evidence supports the novel idea of MND as a multisystem neurodegenerative disease and that action tremor can be part of this condition.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.13743","type":"journal-article","created":{"date-parts":[[2018,6,28]],"date-time":"2018-06-28T18:18:36Z","timestamp":1530209916000},"page":"394","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Tremor in motor neuron disease may be central rather than peripheral in origin"],"prefix":"10.1111","volume":"26","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7103-5209","authenticated-orcid":false,"given":"A.","family":"Latorre","sequence":"first","affiliation":[{"name":"Sobell Department of Motor Neuroscience and Movement Disorders University College London Institute of Neurology London UK"},{"name":"Department of Neurology and Psychiatry Sapienza, University of Rome Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.","family":"Rocchi","sequence":"additional","affiliation":[{"name":"Sobell Department of Motor Neuroscience and Movement Disorders University College London Institute of Neurology London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Stamelou","sequence":"additional","affiliation":[{"name":"Department of Neurology Philipps University Marburg Germany"},{"name":"Parkinson's Disease and Movement Disorders Department HYGEIA Hospital Athens Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Batla","sequence":"additional","affiliation":[{"name":"Sobell Department of Motor Neuroscience and Movement Disorders University College London Institute of Neurology London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Ciocca","sequence":"additional","affiliation":[{"name":"Department of Neurology Fatebenefratelli Hospital ASST Fatebenefratelli Sacco Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B.","family":"Balint","sequence":"additional","affiliation":[{"name":"Sobell Department of Motor Neuroscience and Movement Disorders University College London Institute of Neurology London UK"},{"name":"Department of Neurology University Hospital Heidelberg Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K.","family":"Sidle","sequence":"additional","affiliation":[{"name":"Department of Clinical Neuroscience University College London Institute of Neurology London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3598-3142","authenticated-orcid":false,"given":"A.","family":"Berardelli","sequence":"additional","affiliation":[{"name":"Department of Neurology and Psychiatry Sapienza, University of Rome Rome Italy"},{"name":"Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Neuromed Pozzilli Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. 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