{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,9]],"date-time":"2026-03-09T03:37:21Z","timestamp":1773027441110,"version":"3.50.1"},"reference-count":43,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2025,7,28]],"date-time":"2025-07-28T00:00:00Z","timestamp":1753660800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2025,7,28]],"date-time":"2025-07-28T00:00:00Z","timestamp":1753660800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"University of Innsbruck and Medical University of Innsbruck"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Neural Transm"],"published-print":{"date-parts":[[2026,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Huntington\u00b4s Disease (HD) is an autosomal dominant, neurodegenerative disorder with characteristic motor, behavioural, and cognitive impairment. Mutant Huntingtin may also affect peripheral tissue. We aimed to assess skeletal muscle (SMM) and fat mass, sarcopenia (EWGSOP2), and malnutrition in HD patients in early disease stages compared to age- and sex-matched healthy subjects. Body composition was evaluated by bioelectrical impedance analysis. The Unified HD Rating Scale and cognitive assessments were used for clinical characterization. Twenty early-stage HD patients (45% females) with a median age of 57\u00a0years, body mass index of 22\u00a0kg\/m\n                    <jats:sup>2<\/jats:sup>\n                    , Total Motor Score of 17 points, and Total Functional Capacity of 10 points were included consecutively and prospectively. Confirmed sarcopenia (15%, n\u2009=\u20093) was uncommon. Appendicular SMM index was reduced in 60% (n\u2009=\u200912) and body fat mass in 35% (n\u2009=\u20097). SMM reduction was significantly associated with low weight (p\u2009=\u20090.049) and body fat (p\u2009=\u20090.048). Patients in disease-stage2 (n\u2009=\u200911) had a lower weight (p\u2009=\u20090.009) and body fat (p\u2009=\u20090.008) than patients in disease-stage1 (n\u2009=\u20099; i.e., patients without functional decline). Weight was also lower (p\u2009=\u20090.011) when compared to 20 healthy controls (45% females; median age 56\u00a0years). Fifty-five % of HD patients were at risk for malnutrition or malnourished (Mini Nutritional Assessment). The latter correlated with weight (r\n                    <jats:sub>s<\/jats:sub>\n                    \u2009=\u20090.724, p\u2009&lt;\u20090.001), SMM (r\n                    <jats:sub>s<\/jats:sub>\n                    \u2009=\u20090.473, p\u2009=\u20090.035), body fat mass (r\n                    <jats:sub>s<\/jats:sub>\n                    \u2009=\u20090.611, p\u2009=\u20090.004), motor symptoms (r\n                    <jats:sub>s<\/jats:sub>\n                    \u2009=\u2009\u2212 0.519, p\u2009=\u20090.019), independence (r\n                    <jats:sub>s<\/jats:sub>\n                    \u2009=\u20090.450, p\u2009=\u20090.046), and executive function (r\n                    <jats:sub>s<\/jats:sub>\n                    \u2009=\u20090.526, p\u2009=\u20090.017). Reduction of muscle or fat mass and malnutrition are common even in early-stage HD, which may contribute to progressive wasting and dependence.\n                  <\/jats:p>","DOI":"10.1007\/s00702-025-02991-3","type":"journal-article","created":{"date-parts":[[2025,7,28]],"date-time":"2025-07-28T05:35:55Z","timestamp":1753680955000},"page":"143-153","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Silent destruction: hidden muscle wasting and body decline in early Huntington\u2019s disease"],"prefix":"10.1007","volume":"133","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0791-7300","authenticated-orcid":false,"given":"Marina","family":"Peball","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pia","family":"Sch\u00f6rghuber","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Federico","family":"Carbone","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anne","family":"Zinganell","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Franziska","family":"Di Pauli","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Katar\u00edna","family":"Schwarzov\u00e1","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Atbin","family":"Djamshidian","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Klaus","family":"Seppi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6749-3044","authenticated-orcid":false,"given":"Beatrice","family":"Heim","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2025,7,28]]},"reference":[{"key":"2991_CR1","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2021.595679","volume":"12","author":"D Abreu","year":"2021","unstructured":"Abreu D, Ware J, Georgiou-Karistianis N, Leavitt BR, Fitzer-Attas CJ, Lobo R, Fernandes AR, Handley O, Anderson KE, Stout JC, Sampaio C (2021) Utility of Huntington\u2019s disease assessments by disease stage: floor\/ceiling effects. 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Financial Disclosures for the previous 12\u00a0months: MP received speaking honoraria (lectures) by Candoro ethics Austria. FC, PS, and K Schwarzov\u00e1 have no conflict of interest. AZ has participated in meetings sponsored by, received speaking honoraria or travel funding from Biogen, Merck, Novartis, Sanofi-Genzyme, Janssen and Teva. FDP has participated in meetings sponsored by, received honoraria (lectures, advisory boards, consultations) or travel funding from Amgen, Bayer, Biogen, Janssen, Merck, Neuraxpharm, Novartis, Sanofi-Genzyme, Teva, Celgene and Roche. AD reports honoraria from Novo Nordisk, Roche, Bial, and AbbVie. K Seppi reports honoraria from the International Parkinson and Movement Disorders Society, grants from the FWF Austrian Science Fund, the Michael J. Fox Foundation, and the International Parkinson and Movement Disorder Society, as well as personal fees from Teva, UCB, Lundbeck, AOP Orphan Pharmaceuticals AG, AbbVie, Bial, Roche, and Gr\u00fcnenthal outside the submitted work. BH reports honoraria from Novartis AG, AbbVie, Bial and grants from the Austrian science fund (FWF) outside the submitted work.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}},{"value":"The study protocol was approved by the local ethics committees. All participants gave written informed consent prior to their participation. The interviews and examinations were carried out in accordance with the principles expressed in the Declaration of Helsinki of 1975 and its later amendments. On behalf of all co-authors, the first and corresponding author confirm that all authors have read the Journal\u2019s position on issues involved in ethical publication and affirm that this work is consistent with those guidelines.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval"}},{"value":"Informed consent was obtained from all individual participants included in the study.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent to participate"}}]}}