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      Sporadic Creutzfeldt–Jakob disease presenting as dizziness and cognitive decline : A case report

      case-report

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          Abstract

          Rationale:

          Creutzfeldt–Jakob disease (CJD) is an infrequent and rapidly fatal neurodegenerative disease without effective cure. Common presentations of CJD include rapidly progressive cognitive decline, behavioral changes, cerebellar dysfunction, and visual disturbances. Since clinicians may see only very few cases during their professional career, it is important to be familiar with the clinical presentation and progression, and allow for quick diagnosis.

          Patient concerns:

          We reported an elderly woman had recurrent attacks of dizziness in the preceding month. She began to suffer progression of memory disturbance half a month before admission and was admitted to our department in a coma.

          Diagnosis:

          The accessory examinations of magnetic resonance imaging (MRI), electroencephalography (EEG), 14-3-3 protein in cerebrospinal fluid and S100 protein in serum support the diagnosis of sporadic CJD (sCJD). Combined with this evidence and clinical symptom, we made a clinical diagnosis of sCJD.

          Interventions:

          Supportive treatment.

          Outcomes:

          After 2 months of active treatment, the patient's condition had not improved, and the patient died 82 days after admission.

          Lessons:

          Clinicians should attach importance to sCJD, which is significant for the prevention of transmission and treatment.

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          Most cited references12

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          Creutzfeldt-Jakob disease: updated diagnostic criteria, treatment algorithm, and the utility of brain biopsy.

          Creutzfeldt-Jakob disease (CJD) is a rare neurodegenerative condition with a rapid disease course and a mortality rate of 100%. Several forms of the disease have been described, and the most common is the sporadic type. The most challenging aspect of this disease is its diagnosis-the gold standard for definitive diagnosis is considered to be histopathological confirmation-but newer tests are providing means for an antemortem diagnosis in ways less invasive than brain biopsy. Imaging studies, electroencephalography, and biomarkers are used in conjunction with the clinical picture to try to make the diagnosis of CJD without brain tissue samples, and all of these are reviewed in this article. The current diagnostic criteria are limited; test sensitivity and specificity varies with the genetics of the disease as well as the clinical stage. Physicians may be unsure of all diagnostic testing available, and may order outdated tests or prematurely request a brain biopsy when the diagnostic workup is incomplete. The authors review CJD, discuss the role of brain biopsy in this patient population, provide a diagnostic pathway for the patient presenting with rapidly progressive dementia, and propose newer diagnostic criteria.
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            Prion diseases.

            Prion diseases are degenerative disorders of the nervous system caused by transmissible particles that contain a pathogenic isoform of the prion protein, a normal constituent of cell membranes. The most common human prion disease is Creutzfeldt-Jakob disease (CJD). Most cases are sporadic with unknown mode of transmission, 10-15% of cases are inherited, and a small number have been transmitted by medical procedures. The spread of human prion diseases through consumption of infected material has been implicated historically in kuru and recently in variant CJD. Animal prion diseases (scrapie of sheep, transmissible mink encephalopathy, chronic wasting disease of cervids, and bovine spongiform encephalopathy) all seem to be laterally transmitted by contact with infected animals or by consumption of infected feed. The different modes of transmission of different prion diseases, the unpredictable species barriers, the variable distribution of infectivity in tissues, and strain variations found in some diseases all make risk assessment and predictions of future events difficult.
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              The function of the cellular prion protein in health and disease

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                Author and article information

                Journal
                Medicine (Baltimore)
                Medicine (Baltimore)
                MEDI
                Medicine
                Wolters Kluwer Health
                0025-7974
                1536-5964
                June 2019
                14 June 2019
                : 98
                : 24
                : e16002
                Affiliations
                [a ]Department of Neurology
                [b ]Department of Radiology, The Affiliated Hospital of Jiangsu University, Zhenjiang, Jiangsu, China.
                Author notes
                []Correspondence: Ming Yu, Department of Neurology, The Affiliated Hospital of Jiangsu University, 438 Jiefang Road, Zhenjiang, Jiangsu, 212001, China (e-mail: yuming7251@ 123456163.com ); Yuefeng Li, Department of Radiology, The Affiliated Hospital of Jiangsu University, 438 Jiefang Road, Zhenjiang, Jiangsu, 212001, China (e-mail: jiangdalyf@ 123456126.com ).
                Article
                MD-D-18-09800 16002
                10.1097/MD.0000000000016002
                6587618
                31192942
                42dcc787-b86c-427d-be9d-b2de064e18fa
                Copyright © 2019 the Author(s). Published by Wolters Kluwer Health, Inc.

                This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. http://creativecommons.org/licenses/by-nc-nd/4.0

                History
                : 3 January 2019
                : 23 April 2019
                : 17 May 2019
                Categories
                5300
                Research Article
                Clinical Case Report
                Custom metadata
                TRUE

                14-3-3 protein,creutzfeldt–jakob disease,diffusion weighted imaging,electroencephalography,s100 protein

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