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The aim of this study was to describe the clinical and genetic manifestations of six patients with adult\u2010onset VLCADD.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>In this study, the clinical, pathological and genetic findings of six adult patients (four from Iran and two from Serbia) with VLCADD and their response to treatment are described.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The median (range) age of patients at first visit was 31 (27\u201338) years, and the median (range) age of onset was 26.5 (19\u201333) years. Parental consanguinity was present for four patients. Four patients had a history of rhabdomyolysis, and the recorded CK level ranged between 67 and 90\u00a0000\u00a0IU\/l. Three patients had a history of exertional myalgia, and one patient had a non\u2010fluctuating weakness. Through next\u2010generation sequencing analysis, we identified six cases with variants in the <jats:italic>ACADVL<\/jats:italic> gene and a confirmed diagnosis of VLCADD. Of the total six variants identified, five were missense, and one was a novel frameshift mutation identified in two unrelated individuals. Two variants were novel, and three were previously reported. We treated the patients with a combination of L\u2010carnitine, Coenzyme Q10 and riboflavin. Three patients responded favorably to the treatment.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Adult\u2010onset VLCADD is a rare entity with various presentations. Patients may respond favorably to a cocktail of L\u2010carnitine, Coenzyme Q10, and riboflavin.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.14402","type":"journal-article","created":{"date-parts":[[2020,6,17]],"date-time":"2020-06-17T21:01:05Z","timestamp":1592427665000},"page":"2257-2266","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["Adult\u2010onset very\u2010long\u2010chain acyl\u2010CoA dehydrogenase deficiency (VLCADD)"],"prefix":"10.1111","volume":"27","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6774-7825","authenticated-orcid":false,"given":"F.","family":"Fatehi","sequence":"first","affiliation":[{"name":"Neurology Department Shariati Hospital Tehran University of Medical Sciences Tehran Iran"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9286-4943","authenticated-orcid":false,"given":"A. 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