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However, in the clinical setting, the early identification of patients with possible CJD is often challenging. Indeed, CJD patients may present with isolated symptoms that remain the only clinical manifestation for some time, or with neurological syndromes atypical for CJD. To enhance awareness of unusual disease presentations and promote earlier diagnosis, we reviewed the entire spectrum of atypical early manifestations of CJD, mainly reported to date as case descriptions or small case series. They included sensory either visual or auditory disturbances, seizures, isolated psychiatric manifestations, atypical parkinsonian syndromes (corticobasal syndrome, progressive supranuclear palsy-like), pseudobulbar syndrome, isolated involuntary movements (dystonia, myoclonus, chorea, blepharospasm), acute or subacute onsets mimicking a stroke, isolated aphasia, and neuropathy. Since CJD is a rare disease and its clinical course rapidly progressive, an in-depth understanding and awareness of early clinical features are mandatory to enhance the overall diagnostic accuracy in its very early stages and to recruit optimal candidates for future therapeutic trials.<\/jats:p>","DOI":"10.3233\/jad-180123","type":"journal-article","created":{"date-parts":[[2018,7,13]],"date-time":"2018-07-13T13:39:26Z","timestamp":1531489166000},"page":"1051-1065","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":45,"title":["Unusual Clinical Presentations Challenging the Early Clinical Diagnosis of Creutzfeldt-Jakob Disease"],"prefix":"10.1177","volume":"64","author":[{"given":"Simone","family":"Baiardi","sequence":"first","affiliation":[{"name":"Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabina","family":"Capellari","sequence":"additional","affiliation":[{"name":"Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy"},{"name":"IRCCS Institute of Neurological Sciences, Bologna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Bartoletti Stella","sequence":"additional","affiliation":[{"name":"IRCCS Institute of Neurological Sciences, Bologna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Piero","family":"Parchi","sequence":"additional","affiliation":[{"name":"IRCCS Institute of Neurological Sciences, Bologna, Italy"},{"name":"Department of Experimental, Diagnostic and Specialty Medicine (DIMES), University of Bologna, Bologna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2018,7,12]]},"reference":[{"key":"ref001","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.95.23.13363"},{"key":"ref002","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-010-0779-6"},{"key":"ref003","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-010-0760-4"},{"key":"ref004","doi-asserted-by":"publisher","DOI":"10.3201\/eid1806.120116"},{"key":"ref005","doi-asserted-by":"publisher","DOI":"10.12688\/f1000research.12681.1"},{"key":"ref006","doi-asserted-by":"publisher","DOI":"10.1002\/1531-8249(199908)46:2<224::AID-ANA12>3.0.CO;2-W"},{"key":"ref007","doi-asserted-by":"publisher","DOI":"10.1101\/cshperspect.a024349"},{"key":"ref008","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-014-7410-z"},{"key":"ref009","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-1842.2009.00848.x"},{"key":"ref010","doi-asserted-by":"publisher","DOI":"10.3233\/JAD-150668"},{"key":"ref011","doi-asserted-by":"publisher","DOI":"10.1007\/BF02892896"},{"key":"ref012","doi-asserted-by":"publisher","DOI":"10.1136\/bjo.2005.074856"},{"key":"ref013","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.56.1.55"},{"key":"ref014","doi-asserted-by":"publisher","DOI":"10.1007\/BF03165066"},{"key":"ref015","doi-asserted-by":"publisher","DOI":"10.1111\/jon19977150"},{"key":"ref016","doi-asserted-by":"publisher","DOI":"10.1097\/00041327-200106000-00008"},{"key":"ref017","first-page":"63","volume":"14","author":"Tsuji Y","year":"2004","journal-title":"J Neuroimaging"},{"key":"ref018","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000228224.35678.60"},{"key":"ref019","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000208487.18608.41"},{"key":"ref020","doi-asserted-by":"publisher","DOI":"10.1093\/ageing\/afm191"},{"key":"ref021","doi-asserted-by":"publisher","DOI":"10.3129\/i08-123"},{"key":"ref022","doi-asserted-by":"publisher","DOI":"10.1111\/j.1552-6569.2008.00294.x"},{"key":"ref023","first-page":"48","volume":"12","author":"Kher M","year":"2009","journal-title":"Ann Indian Acad Neurol"},{"key":"ref024","doi-asserted-by":"crossref","unstructured":"VermaR, JunewarV, SahuR (2013) Creutzfeldt-Jakob disease presenting with visual symptoms: A case of the \u2018Heidenhain variant\u2019. 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