{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,3,12]],"date-time":"2025-03-12T04:43:08Z","timestamp":1741754588187,"version":"3.38.0"},"reference-count":8,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2025,1,23]],"date-time":"2025-01-23T00:00:00Z","timestamp":1737590400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2025,4]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Introduction\/Aims<\/jats:title><jats:p>Finger Extension Weakness and DOwnbeat Nystagmus Motor Neuron Disease (FEWDON\u2010MND) is characterized by motor weakness predominantly affecting finger extension, accompanied by downbeat nystagmus. To date, only 11 patients have been reported. The present study adds a further three and aims to provide a more detailed description of the electrodiagnostic features of these patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We present the clinical and electrophysiological features of three French patients from specialized motor neuron centers and review the electrophysiological findings of previously reported patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>These three patients presented with pure motor weakness affecting finger extension and downbeat nystagmus. They exhibited a slowly progressive disease course without respiratory involvement. Nerve conduction studies showed decreased compound muscle action potential amplitudes in the extensor indicis muscles. Abnormal spontaneous activity on needle electromyography (EMG) was rare in two patients, absent in one, and otherwise limited to weak muscles. Additionally, chronic motor axon loss features suggestive of motor neuronopathy were seen in our patients. Importantly, they were also detected in distant asymptomatic muscles.<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>The three patients reported here confirm the typical phenotype of FEWDON\u2010MND, characterized by slowly progressive distal motor weakness initially affecting finger extension, associated with downbeat nystagmus. Although chronic motor axon loss features have been found in all reported patients, our three patients show that active denervation can be absent or rare. Thus, finger drop and diffuse chronic neurogenic changes on EMG should lead clinicians to look for downbeat nystagmus and to consider FEWDON\u2010MND.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mus.28357","type":"journal-article","created":{"date-parts":[[2025,1,23]],"date-time":"2025-01-23T09:20:52Z","timestamp":1737624052000},"page":"644-650","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Electrophysiological Abnormalities in Finger Extension Weakness and <scp>DOwnbeat<\/scp> Nystagmus Motor Neuron Disease: Three New Patients and Review of the Literature"],"prefix":"10.1002","volume":"71","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5694-3759","authenticated-orcid":false,"given":"Julian","family":"Theuriet","sequence":"first","affiliation":[{"name":"Service ENMG et de Pathologies Neuromusculaires, Centre de r\u00e9f\u00e9rence Des Maladies Neuromusculaires PACA\u2010R\u00e9union\u2010Rh\u00f4ne Alpes, H\u00f4pital Neurologique Pierre Wertheimer, Hospices Civils de Lyon  Bron France"},{"name":"Service de Neurologie, Troubles du Mouvement et Pathologies Neuromusculaires, H\u00f4pital Neurologique Pierre\u2010Wertheimer, Hospices Civils de Lyon  Bron France"},{"name":"Centre de r\u00e9f\u00e9rence SLA de Lyon, H\u00f4pital Neurologique Pierre Wertheimer, Hospices Civils de Lyon  Bron France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Emilien","family":"Bernard","sequence":"additional","affiliation":[{"name":"Service ENMG et de Pathologies Neuromusculaires, Centre de r\u00e9f\u00e9rence Des Maladies Neuromusculaires PACA\u2010R\u00e9union\u2010Rh\u00f4ne Alpes, H\u00f4pital Neurologique Pierre Wertheimer, Hospices Civils de Lyon  Bron France"},{"name":"Service de Neurologie, Troubles du Mouvement et Pathologies Neuromusculaires, H\u00f4pital Neurologique Pierre\u2010Wertheimer, Hospices Civils de Lyon  Bron France"},{"name":"Centre de r\u00e9f\u00e9rence SLA de Lyon, H\u00f4pital Neurologique Pierre Wertheimer, Hospices Civils de Lyon  Bron France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nathalie","family":"Guy","sequence":"additional","affiliation":[{"name":"Service de Neurologie, CHU Gabriel Montpied  Clermont\u2010Ferrand France"},{"name":"Centre de Ressource et de comp\u00e9tence Pour la SLA et la Maladie du Neurone Moteur, CHU de Clermont Ferrand, CHU Gabriel Montpied  Clermont Ferrand France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fr\u00e9d\u00e9ric","family":"Taithe","sequence":"additional","affiliation":[{"name":"Service de Neurologie, CHU Gabriel Montpied  Clermont\u2010Ferrand France"},{"name":"Centre de Ressource et de comp\u00e9tence Pour la SLA et la Maladie du Neurone Moteur, CHU de Clermont Ferrand, CHU Gabriel Montpied  Clermont Ferrand France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C\u00e9cilia","family":"Even","sequence":"additional","affiliation":[{"name":"D\u00e9partement de Neurophysiologie Clinique H\u00f4pital Piti\u00e9\u2010Salp\u00eatri\u00e8re, Assistance Publique \u2010 H\u00f4pitaux de Paris  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thierry","family":"Maisonobe","sequence":"additional","affiliation":[{"name":"D\u00e9partement de Neurophysiologie Clinique H\u00f4pital Piti\u00e9\u2010Salp\u00eatri\u00e8re, Assistance Publique \u2010 H\u00f4pitaux de Paris  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Aude","family":"Sangar\u00e9","sequence":"additional","affiliation":[{"name":"D\u00e9partement de Neurophysiologie Clinique H\u00f4pital Piti\u00e9\u2010Salp\u00eatri\u00e8re, Assistance Publique \u2010 H\u00f4pitaux de Paris  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pierre","family":"Lardeux","sequence":"additional","affiliation":[{"name":"Service de Neurocognition et Neuro\u2010Ophtalmologie, H\u00f4pital Neurologique Pierre\u2010Wertheimer, Hospices Civils de Lyon, Groupement Est  Bron France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Caroline Froment","family":"Tilikete","sequence":"additional","affiliation":[{"name":"Service de Neurocognition et Neuro\u2010Ophtalmologie, H\u00f4pital Neurologique Pierre\u2010Wertheimer, Hospices Civils de Lyon, Groupement Est  Bron France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Philippe","family":"Couratier","sequence":"additional","affiliation":[{"name":"Service de Neurologie, CHU de Limoges  Limoges France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Timoth\u00e9e","family":"Lenglet","sequence":"additional","affiliation":[{"name":"D\u00e9partement de Neurophysiologie Clinique H\u00f4pital Piti\u00e9\u2010Salp\u00eatri\u00e8re, Assistance Publique \u2010 H\u00f4pitaux de Paris  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1563-9432","authenticated-orcid":false,"given":"Antoine","family":"Pegat","sequence":"additional","affiliation":[{"name":"Service ENMG et de Pathologies Neuromusculaires, Centre de r\u00e9f\u00e9rence Des Maladies Neuromusculaires PACA\u2010R\u00e9union\u2010Rh\u00f4ne Alpes, H\u00f4pital Neurologique Pierre Wertheimer, Hospices Civils de Lyon  Bron France"},{"name":"Service de Neurologie, Troubles du Mouvement et Pathologies Neuromusculaires, H\u00f4pital Neurologique Pierre\u2010Wertheimer, Hospices Civils de Lyon  Bron France"},{"name":"Centre de r\u00e9f\u00e9rence SLA de Lyon, H\u00f4pital Neurologique Pierre Wertheimer, Hospices Civils de Lyon  Bron 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Nerve"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mus.28357","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,3,11]],"date-time":"2025-03-11T21:55:21Z","timestamp":1741730121000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/mus.28357"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,1,23]]},"references-count":8,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2025,4]]}},"alternative-id":["10.1002\/mus.28357"],"URL":"https:\/\/doi.org\/10.1002\/mus.28357","archive":["Portico"],"relation":{},"ISSN":["0148-639X","1097-4598"],"issn-type":[{"type":"print","value":"0148-639X"},{"type":"electronic","value":"1097-4598"}],"subject":[],"published":{"date-parts":[[2025,1,23]]},"assertion":[{"value":"2024-10-07","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication 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