{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,3]],"date-time":"2026-06-03T00:09:03Z","timestamp":1780445343745,"version":"3.54.1"},"reference-count":12,"publisher":"BMJ","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["BMJ Case Reports"],"abstract":"<jats:p>Creutzfeldt-Jakob disease (CJD) belongs to a group of prion diseases that may be caused by the abnormal folding of proteins called prion proteins. The \u2018Heidenhain variant\u2019 is a subclass of patients with CJD, who present with isolated visual symptoms at the onset without any cognitive decline. Here we report such a case of an elderly man presenting with progressive diminution of vision, forgetfulness, abnormal behaviour, myoclonic jerks and akinetic mutism since the last 5\u2005months. On clinical examination, lead pipe rigidity was present in all four limbs, and plantars were bilateral extensors. In view of rapidly progressive dementia associated with myoclonus, a possibility of CJD was entertained. As visual symptoms preceded dementia, hence the Heidenhain variant was strongly suspected. MRI of the brain revealed cortical ribboning, and EEG showed periodic triphasic waveforms with background slowing. The patient succumbed to the illness within 1\u2005month of hospitalisation.<\/jats:p>","DOI":"10.1136\/bcr-2012-008006","type":"journal-article","created":{"date-parts":[[2013,1,30]],"date-time":"2013-01-30T04:23:41Z","timestamp":1359519821000},"page":"bcr2012008006","source":"Crossref","is-referenced-by-count":8,"title":["Creutzfeldt-Jakob disease presenting with visual symptoms: a case of the \u2018Heidenhain variant\u2019"],"prefix":"10.1136","volume":"2013","author":[{"given":"Rajesh","family":"Verma","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vivek","family":"Junewar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ritesh","family":"Sahu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2013,1,29]]},"reference":[{"key":"2025042701492911000_2013.jan29_1.bcr2012008006.1","doi-asserted-by":"publisher","DOI":"10.4103\/0377-4929.94847"},{"key":"2025042701492911000_2013.jan29_1.bcr2012008006.2","first-page":"131","article-title":"Creutzfeldt-Jakob disease presenting with unusual psychiatric symptoms","volume":"10","author":"Tho","year":"2005","journal-title":"Rep Asian Patient Neurol Asia"},{"key":"2025042701492911000_2013.jan29_1.bcr2012008006.3","doi-asserted-by":"crossref","first-page":"343","DOI":"10.4103\/0971-3026.32302","article-title":"Creutzfeldt-Jakob disease, spongiform encephalopathies, Prion disease, diffusion weighted MRI","volume":"16","author":"Gupta","year":"2006","journal-title":"Indian J Radiol Imaging"},{"key":"2025042701492911000_2013.jan29_1.bcr2012008006.4","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1097\/00041327-200106000-00008","article-title":"The Heidenhain variant of Creutzfeldt\u2013Jakob disease: clinical, pathologic, and neuroimaging Findings","volume":"21","author":"Jacobs","year":"2001","journal-title":"J Neuroophthalmol"},{"key":"2025042701492911000_2013.jan29_1.bcr2012008006.5","doi-asserted-by":"publisher","DOI":"10.1136\/bjo.2005.074856"},{"key":"2025042701492911000_2013.jan29_1.bcr2012008006.6","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.47.2.134"},{"key":"2025042701492911000_2013.jan29_1.bcr2012008006.7","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1097\/WNO.0b013e3181e2aef7","article-title":"MRI and positron emission tomography findings in Heidenhain variant Creutzfeldt-Jakob disease","volume":"30","author":"Prasad","year":"2010","journal-title":"J Neuroophthalmol"},{"key":"2025042701492911000_2013.jan29_1.bcr2012008006.8","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.56.1.55"},{"key":"2025042701492911000_2013.jan29_1.bcr2012008006.9","first-page":"791","article-title":"Creutzfeldt\u2013Jakob disease","volume":"53","author":"Narayan","year":"2005","journal-title":"JAPI"},{"key":"2025042701492911000_2013.jan29_1.bcr2012008006.10","doi-asserted-by":"publisher","DOI":"10.1186\/1757-1626-1-146"},{"key":"2025042701492911000_2013.jan29_1.bcr2012008006.11","first-page":"338","article-title":"CJD: report of 10 cases from North India","volume":"49","author":"Mehndiratta","year":"2001","journal-title":"Neurol India"},{"key":"2025042701492911000_2013.jan29_1.bcr2012008006.12","doi-asserted-by":"publisher","DOI":"10.1159\/000023462"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2012-008006","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,27]],"date-time":"2025-04-27T08:49:32Z","timestamp":1745743772000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2012-008006"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,1,29]]},"references-count":12,"alternative-id":["10.1136\/bcr-2012-008006"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2012-008006","relation":{},"ISSN":["1757-790X"],"issn-type":[{"value":"1757-790X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,1,29]]}}}