{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,17]],"date-time":"2026-07-17T17:12:52Z","timestamp":1784308372382,"version":"3.55.0"},"reference-count":16,"publisher":"BMJ","issue":"3","funder":[{"name":"Nutricia GmbH","award":["N\/A"],"award-info":[{"award-number":["N\/A"]}]}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["J Neurol Neurosurg Psychiatry"],"published-print":{"date-parts":[[2022,3]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>There is growing evidence that the course of amyotrophic lateral sclerosis (ALS) may be influenced beneficially by applying high-caloric food supplements (HCSs). However, it is unknown which composition of nutrients offers optimal tolerability and weight gain.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We conducted a randomised controlled study (Safety and Tolerability of Ultra-high-caloric Food Supplements in Amyotrophic Lateral Sclerosis (ALS); TOLCAL-ALS study) in 64 patients with possible, probable or definite ALS according to El Escorial criteria. Patients were randomised into four groups: a high-caloric fatty supplement (HCFS; 405 kcal\/day, 100% fat), an ultra-high-caloric fatty supplement (UHCFS; 810 kcal\/day, 100% fat), an ultra-high-caloric, carbohydrate-rich supplement (UHCCS; 900 kcal\/day, 49% carbohydrates) and an open control (OC) group without any supplement. The primary endpoint was tolerability. Patients were followed up over 4 weeks.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Gastrointestinal side effects were most frequent in the UHCFS group (75.0%), while loss of appetite was most frequent in the UHCCS group (35.3%). During intervention, patients gained +0.9\u2009kg\/month of body weight (IQR \u22120.9 to 1.5; p=0.03) in the HCFS group and +0.9\u2009kg\/month (IQR \u22120.8 to 2.0; p=0.05) in the UHCFS group. A non-significant trend for weight gain (+0.6\u2009kg\/month (IQR \u22120.3 to 1.9; p=0.08)) was observed in the UHCCS group. Patients in OC group continued to lose body weight (\u22120.5\u2009kg\/month, IQR \u22121.4 to 1.3; p=0.42).<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>The findings suggest that HCSs frequently cause mild to moderate tolerability issues in patients with ALS, most notably gastrointestinal symptoms in high-fat supplements, and loss of appetite in high-carbohydrate supplements. All three HCSs tested are suited to increase body weight.<\/jats:p><\/jats:sec>","DOI":"10.1136\/jnnp-2021-328331","type":"journal-article","created":{"date-parts":[[2022,1,12]],"date-time":"2022-01-12T18:25:20Z","timestamp":1642011920000},"page":"298-302","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":22,"title":["Fat-rich versus carbohydrate-rich nutrition in ALS: a randomised controlled study"],"prefix":"10.1136","volume":"93","clinical-trial-number":[{"clinical-trial-number":"nct04172792","registry":"10.18810\/clinical-trials-gov"}],"author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6352-0909","authenticated-orcid":false,"given":"Johannes","family":"Dorst","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Judith","family":"Doenz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0785-379X","authenticated-orcid":false,"given":"Katharina","family":"Kandler","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jens","family":"Dreyhaupt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1647-6201","authenticated-orcid":false,"given":"Hayrettin","family":"Tumani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1841-954X","authenticated-orcid":false,"given":"Simon","family":"Witzel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joachim","family":"Schuster","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Albert C","family":"Ludolph","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2022,1,12]]},"reference":[{"key":"2022021507552032000_93.3.298.1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.53.5.1059"},{"key":"2022021507552032000_93.3.298.2","doi-asserted-by":"crossref","DOI":"10.1016\/j.jns.2020.117257","article-title":"Hypermetabolism is a reality in amyotrophic lateral sclerosis compared to healthy subjects","volume":"420","author":"Fayemendy","year":"2021","journal-title":"J Neurol Sci"},{"key":"2022021507552032000_93.3.298.3","first-page":"309","article-title":"RNM-01 weight stability is associated with longer survival in amyotrophic lateral sclerosis","volume":"20","author":"Wei","year":"2019","journal-title":"Amyotroph Lateral Scler Frontotemporal Degener"},{"key":"2022021507552032000_93.3.298.4","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2010.211474"},{"key":"2022021507552032000_93.3.298.5","doi-asserted-by":"publisher","DOI":"10.1093\/ajcn\/74.3.328"},{"key":"2022021507552032000_93.3.298.6","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000285080.70324.27"},{"key":"2022021507552032000_93.3.298.7","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2020-323372"},{"key":"2022021507552032000_93.3.298.8","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0402026101"},{"key":"2022021507552032000_93.3.298.9","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(14)60222-1"},{"key":"2022021507552032000_93.3.298.10","unstructured":"Ludolph AC , Dorst J , Dreyhaupt J . Effect of high-caloric nutrition on survival in amyotrophic lateral sclerosis. Ann Neurol 2019;17:25661."},{"key":"2022021507552032000_93.3.298.11","doi-asserted-by":"publisher","DOI":"10.1080\/146608200300079536"},{"key":"2022021507552032000_93.3.298.12","doi-asserted-by":"crossref","unstructured":"Fournier CN , Bedlack R , Quinn C . Development and validation of the Rasch-Built overall amyotrophic lateral sclerosis disability scale (roads). JAMA Neurol 2019;30.","DOI":"10.1001\/jamaneurol.2019.4490"},{"key":"2022021507552032000_93.3.298.13","doi-asserted-by":"crossref","first-page":"497","DOI":"10.1080\/21678421.2019.1621346","article-title":"Loss of appetite is associated with a loss of weight and fat mass in patients with amyotrophic lateral sclerosis","volume":"20","author":"Ngo","year":"2019","journal-title":"Amyotroph Lateral Scler Frontotemporal Degener"},{"key":"2022021507552032000_93.3.298.14","doi-asserted-by":"crossref","first-page":"230","DOI":"10.1002\/mus.26749","article-title":"Loss of appetite in amyotrophic lateral sclerosis is associated with weight loss and decreased calorie consumption independent of dysphagia","volume":"61","author":"Mezoian","year":"2020","journal-title":"Muscle Nerve"},{"key":"2022021507552032000_93.3.298.15","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2015-311387"},{"key":"2022021507552032000_93.3.298.16","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2018-318704"}],"container-title":["Journal of Neurology, Neurosurgery &amp; Psychiatry"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/jnnp-2021-328331","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,2,15]],"date-time":"2022-02-15T17:16:08Z","timestamp":1644945368000},"score":1,"resource":{"primary":{"URL":"https:\/\/jnnp.bmj.com\/lookup\/doi\/10.1136\/jnnp-2021-328331"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,1,12]]},"references-count":16,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2022,2,15]]},"published-print":{"date-parts":[[2022,3]]}},"alternative-id":["10.1136\/jnnp-2021-328331"],"URL":"https:\/\/doi.org\/10.1136\/jnnp-2021-328331","relation":{},"ISSN":["0022-3050","1468-330X"],"issn-type":[{"value":"0022-3050","type":"print"},{"value":"1468-330X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,1,12]]}}}