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In evaluating patients with NBIAs in our centers, we have observed that action\u2010induced dystonic opisthotonus is a common and characteristic feature of NBIAs. Here, we present a case series of patients with NBIAs presenting this feature demonstrated by videos. We suggest that dystonic opisthotonus could be a useful \u201cred flag\u201d for clinicians to suspect NBIAs, and we discuss the differential diagnosis of this feature. This would be particularly useful in identifying patients with NBIAs and no iron accumulation as yet on brain imaging (for example, as in phospholipase A2, group IV (cytosolic, calcium\u2010independent) [<jats:italic>PLA2G6<\/jats:italic>]\u2010related disorders), and it has management implications. \u00a9 2013 The Authors. Movement Disorders published by Wiley Periodicals, Inc. on behalf of International Parkinson and Movement Disorder Society.<\/jats:p>","DOI":"10.1002\/mds.25490","type":"journal-article","created":{"date-parts":[[2013,6,4]],"date-time":"2013-06-04T20:26:53Z","timestamp":1370377613000},"page":"1325-1329","source":"Crossref","is-referenced-by-count":36,"title":["Dystonic opisthotonus: A \u201cred flag\u201d for neurodegeneration with brain iron accumulation syndromes?"],"prefix":"10.1002","volume":"28","author":[{"given":"Maria","family":"Stamelou","sequence":"first","affiliation":[{"name":"Sobell Department of Motor Neuroscience and Movement Disorders University College London (UCL) Institute of Neurology London United Kingdom"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Scarlett C.","family":"Lai","sequence":"additional","affiliation":[{"name":"Division of Movement Disorders Department of Neurology Chang Gung Memorial Hospital at Linkou Medical Center and Chang Gung University Taoyuan Taiwan"},{"name":"Neuroscience Research Center, Chang Gung Memorial Hospital at Linkou Medical Center Taoyuan Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Annu","family":"Aggarwal","sequence":"additional","affiliation":[{"name":"Center for Brain and Nervous Diseases Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute Mumbai India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Susanne A.","family":"Schneider","sequence":"additional","affiliation":[{"name":"Sobell Department of Motor Neuroscience and Movement Disorders University College London (UCL) Institute of Neurology London United Kingdom"},{"name":"Department of Neurology University of Kiel Kiel Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Henry","family":"Houlden","sequence":"additional","affiliation":[{"name":"Department of Molecular Neuroscience UCL Institute of Neurology London United Kingdom"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tu\u2010Hsueh","family":"Yeh","sequence":"additional","affiliation":[{"name":"Division of Movement Disorders Department of Neurology Chang Gung Memorial Hospital at Linkou Medical Center and Chang Gung University Taoyuan Taiwan"},{"name":"Neuroscience Research Center, Chang Gung Memorial Hospital at Linkou Medical Center Taoyuan Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Amit","family":"Batla","sequence":"additional","affiliation":[{"name":"Sobell Department of Motor Neuroscience and Movement Disorders University College London (UCL) Institute of Neurology London United Kingdom"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chin\u2010Song","family":"Lu","sequence":"additional","affiliation":[{"name":"Division of Movement Disorders Department of Neurology Chang Gung Memorial Hospital at Linkou Medical Center and Chang Gung University Taoyuan Taiwan"},{"name":"Neuroscience Research Center, Chang Gung Memorial Hospital at Linkou Medical Center Taoyuan Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mohit","family":"Bhatt","sequence":"additional","affiliation":[{"name":"Center for Brain and Nervous Diseases Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute Mumbai India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kailash P.","family":"Bhatia","sequence":"additional","affiliation":[{"name":"Sobell Department of Motor Neuroscience and Movement Disorders University College London (UCL) Institute of Neurology London United Kingdom"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2013,6,4]]},"reference":[{"key":"e_1_2_5_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.22314"},{"key":"e_1_2_5_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11910-010-0136-0"},{"key":"e_1_2_5_4_1","doi-asserted-by":"publisher","DOI":"10.1097\/WCO.0b013e3283550cac"},{"key":"e_1_2_5_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.spen.2012.03.005"},{"key":"e_1_2_5_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.22211"},{"key":"e_1_2_5_7_1","first-page":"921","article-title":"Contribution a l'etude des syndromes du globe pale. 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