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      Understanding Creutzfeldt-Jakob disease in Iran: a systematic review of case reports

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          Abstract

          Objective

          To systematically review the reported cases of Creutzfeldt-Jakob disease (CJD) in Iran.

          Methods

          A comprehensive literature review of CJD cases in Iran was undertaken using the PubMed®, Scopus® and Google Scholar databases. In addition, the Iranian database MagIran was searched for Persian language reports. Case selection used the following criteria: (i) patients of Iranian origin; (ii) publication in peer-reviewed journals or reputable medical databases; (iii) a definitive diagnosis of CJD based on established diagnostic criteria.

          Results

          Thirteen cases from twelve reports were included in this systematic review. The majority of the cases were female (11 of 13; 84.6%). The mean ± SD age of patients at hospital admission was 59.38 ± 7.44 years. The findings of the case review suggested that the prevalence of CJD in Iran is not fully established. CJD may be misdiagnosed alongside other clinical signs. The most prevalent early indications of the disease were psychiatric and neurological in nature. A considerable delay in diagnosis was observed in some cases and there was a shortage of brain autopsy records.

          Conclusion

          Efforts to improve diagnostic capabilities, promote awareness and establish monitoring systems are necessary for managing the challenges of providing an early diagnosis of CJD in Iran.

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          Most cited references33

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          Antisense oligonucleotides in therapy for neurodegenerative disorders.

          Antisense oligonucleotides are synthetic single stranded strings of nucleic acids that bind to RNA and thereby alter or reduce expression of the target RNA. They can not only reduce expression of mutant proteins by breakdown of the targeted transcript, but also restore protein expression or modify proteins through interference with pre-mRNA splicing. There has been a recent revival of interest in the use of antisense oligonucleotides to treat several neurodegenerative disorders using different approaches to prevent disease onset or halt disease progression and the first clinical trials for spinal muscular atrophy and amyotrophic lateral sclerosis showing promising results. For these trials, intrathecal delivery is being used but direct infusion into the brain ventricles and several methods of passing the blood brain barrier after peripheral administration are also under investigation.
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            Iatrogenic Creutzfeldt-Jakob Disease, Final Assessment

            The book on iatrogenic Creutzfeldt-Jakob disease (CJD) in humans is almost closed. This form of CJD transmission via medical misadventures was first detected in 1974. Today, only occasional CJD cases with exceptionally long incubation periods still appear. The main sources of the largest outbreaks were tissues from human cadavers with unsuspected CJD that were used for dura mater grafts and growth hormone extracts. A few additional cases resulted from neurosurgical instrument contamination, corneal grafts, gonadotrophic hormone, and secondary infections from blood transfusions. Although the final solution to the problem of iatrogenic CJD is still not available (a laboratory test to identify potential donors who harbor the infectious agent), certain other measures have worked well: applying special sterilization of penetrating surgical instruments, reducing the infectious potential of donor blood and tissue, and excluding donors known to have higher than normal risk for CJD.
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              Human stem cell–derived astrocytes replicate human prions in a PRNP genotype–dependent manner

              Krejciova et al. present the first study demonstrating that CJD prions infect human stem cell–derived astrocytes in vitro. This provides a physiologically relevant model for discovery of key molecular pathogenic events of CJD and facilitates the development of future therapies.
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                Author and article information

                Journal
                J Int Med Res
                J Int Med Res
                IMR
                spimr
                The Journal of International Medical Research
                SAGE Publications (Sage UK: London, England )
                0300-0605
                1473-2300
                May 2024
                8 May 2024
                : 52
                : 5
                : 03000605241247706
                Affiliations
                [1 ]Department of Virology, Ringgold 440827, universitySchool of Medicine, Iran University of Medical Sciences; , Tehran, Iran
                [2 ]Department of Microbiology and Microbial Biotechnology, Faculty of Life Sciences and Biotechnology, Shahid Beheshti University, Tehran, Iran
                [3 ]Department of Microbiology, Golestan University of Medical Sciences, Gorgan, Iran
                [4 ]School of Medicine, Ringgold 48432, universityTabriz University of Medical Sciences; , Tabriz, Iran
                Author notes
                [*]Angila Ataei-Pirkooh, Department of Virology, School of Medicine, Iran University of Medical Sciences, Hemat Highway, 14535, Tehran, Iran. Email: Ataei.a@ 123456iums.ac.ir
                Author information
                https://orcid.org/0000-0003-2662-8659
                Article
                10.1177_03000605241247706
                10.1177/03000605241247706
                11080748
                38717041
                ca4489cf-13f8-4958-9030-c2a40b018987
                © The Author(s) 2024

                Creative Commons Non Commercial CC BY-NC: This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License ( https://creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages ( https://us.sagepub.com/en-us/nam/open-access-at-sage).

                History
                : 19 January 2024
                : 31 March 2024
                Categories
                Meta-Analysis and Systematic Review
                Custom metadata
                ts2

                creutzfeldt-jakob disease,prion diseases,clinical characteristics,neurodegenerative diseases,case reports

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