{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,6]],"date-time":"2025-10-06T18:35:11Z","timestamp":1759775711328,"version":"3.44.0"},"reference-count":16,"publisher":"BMJ","issue":"3","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2019,2,15]]},"published-print":{"date-parts":[[2019,3]]},"abstract":"<jats:p>Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare neurodegenerative disease that can mimic other neurological disorders. We present a case of sCJD in a 64-year-old man that presented with corticobasal syndrome and survived for 3 years. He presented initially with dementia, hemiparkinsonism and alien limb phenomenon and was diagnosed with corticobasal degeneration, ultimately progressing to immobility and akinetic mutism. With a normal MRI 1\u2009year before onset, his neuroimaging 1\u2009year later revealed abnormal DaTscan, cortical and hippocampal atrophy with ventricular dilatation on MRI, and diffusion-weighted cortical ribboning and thalamic hyperintensity. Postmortem, the patient\u2019s brain was collected by the Parkinson\u2019s UK Tissue Bank. Prion protein immunohistochemistry revealed widespread diffuse microvacuolar staining without kuru-type plaques. Hyperphosphorylated tau was only found in the entorhinal cortex and hippocampus. This case highlights the clinical heterogeneity of sCJD presentation and the important inclusion of CJD in the differential diagnosis of atypical presentations of neurodegenerative disease.<\/jats:p>","DOI":"10.1136\/bcr-2018-228305","type":"journal-article","created":{"date-parts":[[2019,3,8]],"date-time":"2019-03-08T05:04:34Z","timestamp":1552021474000},"page":"e228305","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":9,"title":["Rare histotype of sporadic Creutzfeldt-Jakob disease, clinically suspected as corticobasal degeneration"],"prefix":"10.1136","volume":"12","author":[{"given":"Bension Shlomo","family":"Tilley","sequence":"first","affiliation":[{"name":"Department of Medicine, Imperial College London, London, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Colin","family":"Smith","sequence":"additional","affiliation":[{"name":"National Creutzfeldt-Jakob Disease Research and Surveillance Unit, University of Edinburgh, Edinburgh, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nicola","family":"Pavese","sequence":"additional","affiliation":[{"name":"Institute of Neuroscience, Newcastle University, Newcastle Upon Tyne, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Johannes","family":"Attems","sequence":"additional","affiliation":[{"name":"Institute of Neuroscience, Newcastle University, Newcastle Upon Tyne, 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