{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T15:16:53Z","timestamp":1790090213726,"version":"4.0.1"},"reference-count":26,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2025,6,16]],"date-time":"2025-06-16T00:00:00Z","timestamp":1750032000000},"content-version":"vor","delay-in-days":15,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2025,6]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Neurodegenerative Langerhans Cell Histiocytosis (ND\u2010LCH) is a severe central nervous system involvement complicating LCH. ND\u2010LCH is characterized by a cerebellar ataxia, pyramidal signs, pseudobulbar palsy, cognitive impairment, and behavioral disturbances. Cerebellar atrophy, the most common MRI finding, has been widely described in the literature. However, the natural history of volumetric changes in the cerebellum has never been examined. In this study, we aim to perform a quantitative analysis of cerebellar atrophy in ND\u2010LCH patients compared to a control cohort.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>MRI of patients with ND\u2010LCH was compared to those of controls. An automated cerebellum analysis was performed using the Volbrain software (CERES) and a linear regression analysis was used to score the severity of cerebellar atrophy over time.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We retrospectively analyzed the follow\u2010up MRI of 16 adult patients with ND\u2010LCH compared to those of 22 control patients. We examined an average of four MRIs per patient over a median follow\u2010up period of six years. All patients with ND\u2010LCH exhibit global and cortical cerebellar atrophy over time. The average atrophy rate is \u22121.86\u2009cm<jats:sup>3<\/jats:sup>\/year (range\u2009\u2212\u20095.90 to 0.34) and is heterogeneous between patients. All cerebellar lobules are affected by atrophy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Longitudinal quantitative MRI analysis of the cerebellum in ND\u2010LCH: (i) is feasible, (ii) confirms significant global and cortical cerebellar atrophy in patients with ND\u2010LCH over time, (iii) estimates the rate of cerebellar atrophy, and (iv) could be useful as a therapeutic endpoint in a clinically slow\u2010progressing disease. Further studies are needed to validate our findings.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.70249","type":"journal-article","created":{"date-parts":[[2025,6,16]],"date-time":"2025-06-16T13:41:18Z","timestamp":1750081278000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Quantitative Brain <scp>MRI<\/scp> Analysis in Neurodegenerative Langerhans Cell Histiocytosis"],"prefix":"10.1111","volume":"32","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2014-2762","authenticated-orcid":false,"given":"Chooyoung","family":"Baek","sequence":"first","affiliation":[{"name":"Sorbonne Universit\u00e9, AP\u2010HP, Institut du Cerveau \u2010 Paris Brain Institute \u2010 ICM, Inserm, CNRS, H\u00f4pitaux Universitaires La Piti\u00e9 Salp\u00eatri\u00e8re \u2010 Charles Foix DMU Neurosciences, Service de Neuro\u2010Oncologie\u2010Institut de Neurologie  Paris 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