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Presumed diagnosis was Wernicke\u2019s encephalopathy and intravenous thiamine was given, resulting in partial improvement. Subsequent examination revealed hyperekplexia with ocular flutter, cervical dystonia and startle myoclonus. Cerebrospinal fluid indirect immunofluorescence on primate cerebellum revealed granular layer pattern of staining, prompting testing for anti-dipeptidyl-peptidase-like protein antibodies, which returned strongly positive. Given the phenotypic overlap, it is important to consider this rare disorder in the differential diagnosis of Wernicke\u2019s encephalopathy. In this case, the emergence of ocular flutter, startle myoclonus and the specific type of nystagmus made it clear there was an alternative diagnosis.<\/jats:p>","DOI":"10.1136\/bcr-2024-262573","type":"journal-article","created":{"date-parts":[[2025,1,3]],"date-time":"2025-01-03T01:30:15Z","timestamp":1735867815000},"page":"e262573","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":4,"title":["DPPX antibody-mediated disease mimicking Wernicke\u2019s encephalopathy"],"prefix":"10.1136","volume":"18","author":[{"given":"Yasmin","family":"Soliman","sequence":"first","affiliation":[{"name":"Neurology, Sir Charles Gairdner Hospital, Nedlands, Western Australia, Australia"},{"name":"Neurology, Joondalup Health Campus, Joondalup, Western Australia, Australia"},{"name":"Neuro-opthalmology, Sir Charles Gairdner Hospital, Nedlands, Western Australia, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Neha","family":"Irani","sequence":"additional","affiliation":[{"name":"Neurology, Joondalup Health Campus, Joondalup, Western Australia, Australia"},{"name":"Neuro-opthalmology, Sir Charles Gairdner Hospital, Nedlands, Western Australia, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kevin","family":"O'Connor","sequence":"additional","affiliation":[{"name":"Neurology, Joondalup Health Campus, Joondalup, Western Australia, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2025,1,2]]},"reference":[{"key":"2025082710450361000_18.1.e262573.1","doi-asserted-by":"crossref","first-page":"1340","DOI":"10.1212\/WNL.0000000000003796","article-title":"DPPX antibody-associated encephalitis: Main syndrome and antibody effects","volume":"88","author":"Hara","year":"2017","journal-title":"Neurology (ECronicon)"},{"key":"2025082710450361000_18.1.e262573.2","doi-asserted-by":"crossref","first-page":"890","DOI":"10.1212\/WNL.0000000000001907","article-title":"Anti-DPPX encephalitis: pathogenic effects of antibodies on gut and brain neurons","volume":"85","author":"Piepgras","year":"2015","journal-title":"Neurology (ECronicon)"},{"key":"2025082710450361000_18.1.e262573.3","doi-asserted-by":"crossref","first-page":"1797","DOI":"10.1212\/WNL.0000000000000991","article-title":"DPPX potassium channel antibody: frequency, clinical accompaniments, and outcomes in 20 patients","volume":"83","author":"Tobin","year":"2014","journal-title":"Neurology (ECronicon)"},{"key":"2025082710450361000_18.1.e262573.4","doi-asserted-by":"publisher","DOI":"10.1002\/ana.23756"},{"key":"2025082710450361000_18.1.e262573.5","doi-asserted-by":"crossref","first-page":"1065","DOI":"10.1016\/j.mayocp.2019.02.018","article-title":"Wernicke Encephalopathy-Clinical Pearls","volume":"94","author":"Sinha","year":"2019","journal-title":"Mayo Clin Proc"},{"key":"2025082710450361000_18.1.e262573.6","unstructured":"Victor M . 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