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We describe a purely neurologic allelic form of the disease consisting of deafness, putamenal hyperintensity on MRI and a myoclonic\u2010dystonic movement disorder unchanging from childhood into, so far, the late fourth decade. We show that succinate supplementation circumvents the Krebs cycle block, but does not correct the neurologic disease. Our patients\u2019 Arg407Trp mutation has been reported in children with (yet) no MRI abnormalities. It remains possible that early succinate supplementation could impact the disease.<\/jats:p>","DOI":"10.1002\/acn3.51247","type":"journal-article","created":{"date-parts":[[2020,11,25]],"date-time":"2020-11-25T02:33:30Z","timestamp":1606271610000},"page":"252-258","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["SUCLA2 Arg407Trp mutation can cause a nonprogressive movement disorder \u2013 deafness syndrome"],"prefix":"10.1002","volume":"8","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7965-2532","authenticated-orcid":false,"given":"Reem A.","family":"Alkhater","sequence":"first","affiliation":[{"name":"Program in Genetics and Genome Biology The Hospital for Sick Children Toronto Ontario Canada"},{"name":"Institute of Medical Sciences University of Toronto Toronto Ontario Canada"},{"name":"Johns\u2019 Hopkins Aramco Healthcare Dhahran Saudi 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