{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,6]],"date-time":"2026-05-06T05:36:35Z","timestamp":1778045795957,"version":"3.51.4"},"reference-count":13,"publisher":"BMJ","issue":"3","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["Pract Neurol"],"accepted":{"date-parts":[[2023,11,15]]},"published-print":{"date-parts":[[2024,6]]},"abstract":"<jats:p>We describe an unusual case of clinical amyotrophic lateral sclerosis (ALS) with initial neurophysiological studies suggesting demyelination, along with neuroimaging findings that helped to support the eventual diagnosis. An otherwise well 68-year-old man had 8 weeks of left upper limb weakness. On examination, there were widespread lower and upper motor neurone findings suggesting ALS. However, nerve conduction studies identified sensorimotor demyelinating changes suggesting chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), a diagnosis further supported by cerebrospinal fluid analysis. MR scan of the brain revealed a \u2018motor band\u2019, a feature seen commonly in ALS. His condition was refractory to immunotherapy with clinical progression in-keeping with ALS, establishing the diagnosis. ALS is rarely associated with demyelinating neurophysiological changes resembling CIDP. The clinical phenotype is crucial to support the correct diagnosis and imaging findings may help.<\/jats:p>","DOI":"10.1136\/pn-2023-003963","type":"journal-article","created":{"date-parts":[[2023,12,2]],"date-time":"2023-12-02T13:20:52Z","timestamp":1701523252000},"page":"219-222","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":3,"title":["Amyotrophic lateral sclerosis with demyelinating neurophysiology and a motor band sign"],"prefix":"10.1136","volume":"24","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9438-0697","authenticated-orcid":false,"given":"Matthew","family":"Megens","sequence":"first","affiliation":[{"name":"Department of Neurology, Launceston General Hospital, Launceston, Tasmania, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Aaron","family":"de Souza","sequence":"additional","affiliation":[{"name":"Department of Neurology, Launceston General Hospital, Launceston, Tasmania, Australia"},{"name":"University of Tasmania Launceston Clinical School, Launceston, Tasmania, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2023,12,2]]},"reference":[{"key":"2025090410462568000_24.3.219.1","doi-asserted-by":"crossref","DOI":"10.3389\/fneur.2022.947347","article-title":"Structural magnetic resonance imaging findings and histopathological correlations in motor neuron diseases\u2014A systematic review and meta-analysis","volume":"13","author":"Zejlon","year":"2022","journal-title":"Front Neurol"},{"key":"2025090410462568000_24.3.219.2","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinph.2007.09.143"},{"key":"2025090410462568000_24.3.219.3","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinph.2020.04.005"},{"key":"2025090410462568000_24.3.219.4","doi-asserted-by":"publisher","DOI":"10.1002\/mus.21010"},{"key":"2025090410462568000_24.3.219.5","doi-asserted-by":"crossref","first-page":"356","DOI":"10.1002\/mus.20475","article-title":"A study of three patients with Amyotrophic lateral sclerosis and a polyneuropathy resembling CIDP","volume":"33","author":"Echaniz-Laguna","year":"2006","journal-title":"Muscle Nerve"},{"key":"2025090410462568000_24.3.219.6","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(94)90191-0"},{"key":"2025090410462568000_24.3.219.7","doi-asserted-by":"publisher","DOI":"10.1111\/j.1085-9489.2005.10302.x"},{"key":"2025090410462568000_24.3.219.8","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000286948.99150.16"},{"key":"2025090410462568000_24.3.219.9","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1016\/j.neucli.2013.05.001","article-title":"Rapidly progressive Amyotrophic lateral sclerosis initially masquerading as a Demyelinating neuropathy","volume":"43","author":"Ahdab","year":"2013","journal-title":"Neurophysiol Clin"},{"key":"2025090410462568000_24.3.219.10","doi-asserted-by":"crossref","first-page":"937","DOI":"10.1002\/(SICI)1097-4598(199907)22:7<937::AID-MUS19>3.0.CO;2-6","article-title":"Amplitude\u2010Dependent slowing of conduction in Amyotrophic lateral sclerosis and polyneuropathy","volume":"22","author":"Feinberg","year":"1999","journal-title":"Muscle Nerve"},{"key":"2025090410462568000_24.3.219.11","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000437301.28441.7e"},{"key":"2025090410462568000_24.3.219.12","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0035241","article-title":"Iron accumulation in deep cortical layers accounts for MRI signal abnormalities in ALS: correlating 7 Tesla MRI and pathology","volume":"7","author":"Kwan","year":"2012","journal-title":"PLoS One"},{"key":"2025090410462568000_24.3.219.13","doi-asserted-by":"crossref","DOI":"10.1016\/j.jns.2019.116440","article-title":"The motor band sign in ALS: presentations and frequencies in a consecutive series of ALS patients","volume":"406","author":"Roeben","year":"2019","journal-title":"J Neurol Sci"}],"container-title":["Practical Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/pn-2023-003963","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,9,4]],"date-time":"2025-09-04T17:46:33Z","timestamp":1757007993000},"score":1,"resource":{"primary":{"URL":"https:\/\/pn.bmj.com\/lookup\/doi\/10.1136\/pn-2023-003963"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,12,2]]},"references-count":13,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2024,5,29]]},"published-print":{"date-parts":[[2024,6]]}},"alternative-id":["10.1136\/pn-2023-003963"],"URL":"https:\/\/doi.org\/10.1136\/pn-2023-003963","relation":{},"ISSN":["1474-7758","1474-7766"],"issn-type":[{"value":"1474-7758","type":"print"},{"value":"1474-7766","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,12,2]]}}}