{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,11]],"date-time":"2025-09-11T17:49:29Z","timestamp":1757612969684,"version":"3.44.0"},"reference-count":3,"publisher":"BMJ","issue":"5","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["Pract Neurol"],"accepted":{"date-parts":[[2019,2,18]]},"published-print":{"date-parts":[[2019,10]]},"abstract":"<jats:p>Atypical motor neurone disease (MND) represents a challenging and expanding group of neurodegenerative disorders involving the upper or lower motor neurones, and rarely both. Neuro-ophthalmological disturbances such as gaze-evoked downbeat nystagmus are extremely rare in the context of typical and atypical MND. Finger extension weakness and downbeat nystagmus motor neurone disease (FEWDON-MND) syndrome has been recently recognised as a distinct syndromic phenotype of MND, with a characteristic clinical picture. We describe a 63-year-old woman with long-standing lower motor neurone involvement of the upper limbs, who on examination had gaze-evoked downbeat nystagmus. After extensive negative investigation for secondary causes of MND and downbeat nystagmus, we diagnosed FEWDON-MND syndrome.<\/jats:p>","DOI":"10.1136\/practneurol-2018-002188","type":"journal-article","created":{"date-parts":[[2019,4,24]],"date-time":"2019-04-24T17:21:41Z","timestamp":1556126501000},"page":"424-426","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":5,"title":["Finger extension weakness and downbeat nystagmus motor neurone disease (FEWDON-MND)"],"prefix":"10.1136","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0150-525X","authenticated-orcid":false,"given":"Wladimir B V R","family":"Pinto","sequence":"first","affiliation":[{"name":"Neurology and Neurosurgery, UNIFESP - Federal University of S\u00e3o Paulo, Sao Paulo, Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Igor Braga","family":"Farias","sequence":"additional","affiliation":[{"name":"Neurology and Neurosurgery, UNIFESP - Federal University of S\u00e3o Paulo, Sao Paulo, Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bruno de Mattos Lombardi","family":"Badia","sequence":"additional","affiliation":[{"name":"Neurology and Neurosurgery, UNIFESP - Federal University of S\u00e3o Paulo, Sao Paulo, Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luiz Henrique","family":"Libardi Silva","sequence":"additional","affiliation":[{"name":"Neurology and Neurosurgery, UNIFESP - Federal University of S\u00e3o Paulo, Sao Paulo, Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mario Teruo","family":"Yanagiura","sequence":"additional","affiliation":[{"name":"Neurology and Neurosurgery, UNIFESP - Federal University of S\u00e3o Paulo, Sao Paulo, Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marco Ant\u00f4nio Troccoli","family":"Chieia","sequence":"additional","affiliation":[{"name":"Neurology and Neurosurgery, UNIFESP - Federal University of S\u00e3o Paulo, Sao Paulo, Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paulo Victor Sgobbi de","family":"Souza","sequence":"additional","affiliation":[{"name":"Neurology and Neurosurgery, UNIFESP - Federal University of S\u00e3o Paulo, Sao Paulo, Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Acary Souza Bulle","family":"Oliveira","sequence":"additional","affiliation":[{"name":"Neurology and Neurosurgery, UNIFESP - Federal University of S\u00e3o Paulo, Sao Paulo, Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2019,4,24]]},"reference":[{"key":"2025090409514354000_19.5.424.1","doi-asserted-by":"crossref","unstructured":"Thakore NJ , Pioro EP , Rucker JC , et al . Motor neuronopathy with dropped hands and downbeat nystagmus: a distinctive disorder? A case report. BMC Neurol 2006;6.doi:10.1186\/1471-2377-6-3","DOI":"10.1186\/1471-2377-6-3"},{"key":"2025090409514354000_19.5.424.2","doi-asserted-by":"crossref","first-page":"1164","DOI":"10.1002\/mus.25669","article-title":"Finger extension weakness and downbeat nystagmus motor neuron disease syndrome: a novel motor neuron disorder?","volume":"56","author":"Delva","year":"2017","journal-title":"Muscle Nerve"},{"key":"2025090409514354000_19.5.424.3","doi-asserted-by":"publisher","DOI":"10.1001\/archneurol.2011.130"}],"container-title":["Practical Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/practneurol-2018-002188","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,9,4]],"date-time":"2025-09-04T16:51:48Z","timestamp":1757004708000},"score":1,"resource":{"primary":{"URL":"https:\/\/pn.bmj.com\/lookup\/doi\/10.1136\/practneurol-2018-002188"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,4,24]]},"references-count":3,"journal-issue":{"issue":"5","published-online":{"date-parts":[[2019,10,1]]},"published-print":{"date-parts":[[2019,10]]}},"alternative-id":["10.1136\/practneurol-2018-002188"],"URL":"https:\/\/doi.org\/10.1136\/practneurol-2018-002188","relation":{},"ISSN":["1474-7758","1474-7766"],"issn-type":[{"type":"print","value":"1474-7758"},{"type":"electronic","value":"1474-7766"}],"subject":[],"published":{"date-parts":[[2019,4,24]]}}}