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To date, there have been no detailed neurological descriptions of an adult onset presentation of NAGS deficiency. In this review we examine the clinical presentation and management of UCDs with an emphasis on NAGS deficiency. An illustrative case is provided. Plasma ammonia levels should be measured in all adult patients with unexplained encephalopathy, as treatment can be potentially life-saving. Availability of N-carbamylglutamate (NCG; carglumic acid) has made protein restriction largely unnecessary in treatment regimens currently employed. Genetic counselling remains an essential component of management of NAGS.<\/jats:p>","DOI":"10.1017\/s0317167100012877","type":"journal-article","created":{"date-parts":[[2014,12,12]],"date-time":"2014-12-12T14:58:48Z","timestamp":1418396328000},"page":"3-9","source":"Crossref","is-referenced-by-count":15,"title":["Recurrent Encephalopathy: NAGS (N-Acetylglutamate Synthase) Deficiency in Adults"],"prefix":"10.1017","volume":"40","author":[{"given":"A.","family":"Cartagena","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.N.","family":"Prasad","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C.A.","family":"Rupar","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Strong","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Tuchman","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"N. 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