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Moreover, whether retinal atrophy has a clinical (diagnostic and prognostic) value in these diseases remains unclear.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Objective<\/jats:title>\n                <jats:p>To add light on the pathological significance and clinical value of retinal atrophy in patients with amyotrophic lateral sclerosis (ALS) and Kennedy\u2019s disease (KD).<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>Thirty-five ALS, thirty-seven KD, and forty-nine age-matched healthy controls (HC) were included in a one-year longitudinal study. Spectrum-domain optical coherence tomography (OCT) was performed at study entry (T0) and after 12\u00a0months (T1). Disease duration and functional rating scale (FRS) for ALS and KD patients were correlated to retinal thicknesses.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>Compared to HC, peripapillary retinal nerve fiber layer (pRNFL) thickness was significantly thinner in both ALS (p\u2009=\u20090.034) and KD (p\u2009=\u20090.003). pRNFL was thinner in KD compared to ALS, but the difference was not significant. In KD, pRNFL atrophy significantly correlated with both disease severity (r\u2009=\u20090.296, p\u2009=\u20090.035) and disease duration (r\u2009=\u2009\u2013\u20090.308, p\u2009=\u20090.013) while no significant correlation was found in ALS (disease severity: r\u2009=\u20090.147, p\u2009=\u20090.238; disease duration: r\u2009=\u2009\u2013\u20090.093, p\u2009=\u20090.459). During the follow-up, pRNFL thickness remained stable in KD while significantly decreased in ALS (p\u2009=\u20090.043).<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>Our study provides evidence of retinal atrophy in both ALS and KD and suggests that retinal thinning is a primary local phenomenon in motoneuron diseases. The clinical value of pRNFL atrophy in KD is worthy of further investigation.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1007\/s00415-023-11802-2","type":"journal-article","created":{"date-parts":[[2023,6,8]],"date-time":"2023-06-08T05:01:45Z","timestamp":1686200505000},"page":"4478-4486","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Neurodegeneration in the retina of motoneuron diseases: a longitudinal study in amyotrophic lateral sclerosis and Kennedy\u2019s disease"],"prefix":"10.1007","volume":"270","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7465-6246","authenticated-orcid":false,"given":"Alessandro","family":"Miscioscia","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Puthenparampil","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lorenzo","family":"Blasi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Rinaldi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paola","family":"Perini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gianni","family":"Sorar\u00f9","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paolo","family":"Gallo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2023,6,8]]},"reference":[{"key":"11802_CR1","doi-asserted-by":"publisher","first-page":"17071","DOI":"10.1038\/nrdp.2017.71","volume":"3","author":"O Hardiman","year":"2017","unstructured":"Hardiman O, Al-Chalabi A, Chio A et al (2017) Amyotrophic lateral sclerosis. 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