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Sporadic Creutzfeldt - Jakob Disease: Case Report from a Neuroscience Institute in Bangladesh

Received: 13 August 2021     Accepted: 3 September 2021     Published: 26 September 2021
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Abstract

Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare neurodegenerative disorder characterized by rapidly progressive dementia and myoclonus. Additional clinical features include visual disturbances, cerebellar, and pyramidal/extrapyramidal signs. The rarity of this disease and the wide variety of initial symptoms make the early diagnosis quite challenging. Nevertheless, it can also be diagnosed in a centre with limited resources through good clinical analysis. Here a case of a 53-year-old man is presented who had a 6 weeks history of cognitive deterioration and myoclonus. Brain magnetic resonance imaging (MRI) showed ribbon-like areas of hyperintensity in the bilateral cortex on diffusion-weighted imaging (DWI); bilateral hyperintense caudate nucleus and putamen on T2-weighted and Fluid-attenuated inversion recovery (FLAIR) images. Electroencephalogram (EEG) showed intermittent generalized periodic sharp waves of triphasic morphology. The diagnosis of probable sCJD was reached based on the clinical features and characteristic findings in his MRI and EEG according to WHO criteria. Several other works of the literature were also reviewed for early diagnosis of sCJD and for the exclusion of other differential diagnoses which may mimic sCJD.

Published in Clinical Neurology and Neuroscience (Volume 5, Issue 3)
DOI 10.11648/j.cnn.20210503.16
Page(s) 63-67
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2021. Published by Science Publishing Group

Keywords

Creutzfeldt-Jakob Disease, Prion Disease, Myoclonus, Rapidly Progressing Dementia

References
[1] Department of Health and Human Services, Centers for Disease Control and Prevention, CJD (Creutzfeldt-Jakob Disease, Classic), author 2010. [June 1st 2012]. http://www.cdc.gov/ncidod/dvrd/cjd/.
[2] Rabinovici G. D., Wang M P. N., Levin J., Cook L., Pravdin M., Davis J., et al. First symptom in sporadic Creutzfeldt–Jakobdisease [Internet]. 2006. Available from: www.neurology.org.
[3] Gambetti P, Kong Q, Zou W, Parchi P, Chen SG. Sporadic and familial CJD: classification and characterisation. Available from: https://academic.oup.com/bmb/article/66/1/213/284818
[4] Kharel H, Adhikari P, Pokhrel NB, Kharel Z, Nepal G. The first reported case of Creutzfeldt-Jakob disease from Nepal. Clinical Case Reports. 2020 Jan 1; 8 (1): 198–202.
[5] World Health Organization Emerging and other Communicable Diseases, Surveillance and Control [Internet]. Available from: http://www.who.int/emc.
[6] Kwon GT, Kwon MS. Diagnostic challenge of rapidly progressing sporadic Creutzfeldt-Jakob disease. BMJ Case Reports. 2019 Sep 1; 12 (9).
[7] Xu Y, Xu J, Zhang J, Cai Z, Wei H, Yu M, et al. Sporadic Creutzfeldt-Jakob disease presenting as dizziness and cognitive decline: A case report. Medicine (United States). 2019 Jun 1; 98 (24).
[8] Dirzius E, Balnyte R, Steibliene V, Gleizniene R, Gudinaviciene I, Radziunas A, et al. Sporadic Creutzfeldt-Jakob disease with unusual initial presentation as posterior reversible encephalopathy syndrome: A case report. BMC Neurology. 2016 Nov 22; 16 (1).
[9] Muayqil T, Gronseth G, Camicioli R. Evidence-based guideline: Diagnostic accuracy of CSF 14-3-3 protein in sporadic Creutzfeldt-Jakob disease Report of the Guideline Development Subcommittee of the American Academy of Neurology [Internet]. 2012. Available from: www.neurology.org.
[10] Rentz CA. Creutzfeldt-Jakob disease: Two case studies. American Journal of Alzheimer’s Disease and Other Dementias. 2003; 18 (may): 171–80.
[11] Jardri R, DiPaola C, Lajugie C, Thomas P, Goeb JL. Depressive disorder with psychotic symptoms as psychiatric presentation of sporadic Creutzfeldt-Jakob disease: a case report. General Hospital Psychiatry. 2006 Sep; 28 (5): 452–4.
Cite This Article
  • APA Style

    Tareq Esteak, Mohammad Bazlur Rashid, Md. Ashrafuzzaman Khan, Mohammad Nur Uddin, Mashfiqul Hasan, et al. (2021). Sporadic Creutzfeldt - Jakob Disease: Case Report from a Neuroscience Institute in Bangladesh. Clinical Neurology and Neuroscience, 5(3), 63-67. https://doi.org/10.11648/j.cnn.20210503.16

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    ACS Style

    Tareq Esteak; Mohammad Bazlur Rashid; Md. Ashrafuzzaman Khan; Mohammad Nur Uddin; Mashfiqul Hasan, et al. Sporadic Creutzfeldt - Jakob Disease: Case Report from a Neuroscience Institute in Bangladesh. Clin. Neurol. Neurosci. 2021, 5(3), 63-67. doi: 10.11648/j.cnn.20210503.16

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    AMA Style

    Tareq Esteak, Mohammad Bazlur Rashid, Md. Ashrafuzzaman Khan, Mohammad Nur Uddin, Mashfiqul Hasan, et al. Sporadic Creutzfeldt - Jakob Disease: Case Report from a Neuroscience Institute in Bangladesh. Clin Neurol Neurosci. 2021;5(3):63-67. doi: 10.11648/j.cnn.20210503.16

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  • @article{10.11648/j.cnn.20210503.16,
      author = {Tareq Esteak and Mohammad Bazlur Rashid and Md. Ashrafuzzaman Khan and Mohammad Nur Uddin and Mashfiqul Hasan and Paritosh Kumar Sarkar},
      title = {Sporadic Creutzfeldt - Jakob Disease: Case Report from a Neuroscience Institute in Bangladesh},
      journal = {Clinical Neurology and Neuroscience},
      volume = {5},
      number = {3},
      pages = {63-67},
      doi = {10.11648/j.cnn.20210503.16},
      url = {https://doi.org/10.11648/j.cnn.20210503.16},
      eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.cnn.20210503.16},
      abstract = {Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare neurodegenerative disorder characterized by rapidly progressive dementia and myoclonus. Additional clinical features include visual disturbances, cerebellar, and pyramidal/extrapyramidal signs. The rarity of this disease and the wide variety of initial symptoms make the early diagnosis quite challenging. Nevertheless, it can also be diagnosed in a centre with limited resources through good clinical analysis. Here a case of a 53-year-old man is presented who had a 6 weeks history of cognitive deterioration and myoclonus. Brain magnetic resonance imaging (MRI) showed ribbon-like areas of hyperintensity in the bilateral cortex on diffusion-weighted imaging (DWI); bilateral hyperintense caudate nucleus and putamen on T2-weighted and Fluid-attenuated inversion recovery (FLAIR) images. Electroencephalogram (EEG) showed intermittent generalized periodic sharp waves of triphasic morphology. The diagnosis of probable sCJD was reached based on the clinical features and characteristic findings in his MRI and EEG according to WHO criteria. Several other works of the literature were also reviewed for early diagnosis of sCJD and for the exclusion of other differential diagnoses which may mimic sCJD.},
     year = {2021}
    }
    

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  • TY  - JOUR
    T1  - Sporadic Creutzfeldt - Jakob Disease: Case Report from a Neuroscience Institute in Bangladesh
    AU  - Tareq Esteak
    AU  - Mohammad Bazlur Rashid
    AU  - Md. Ashrafuzzaman Khan
    AU  - Mohammad Nur Uddin
    AU  - Mashfiqul Hasan
    AU  - Paritosh Kumar Sarkar
    Y1  - 2021/09/26
    PY  - 2021
    N1  - https://doi.org/10.11648/j.cnn.20210503.16
    DO  - 10.11648/j.cnn.20210503.16
    T2  - Clinical Neurology and Neuroscience
    JF  - Clinical Neurology and Neuroscience
    JO  - Clinical Neurology and Neuroscience
    SP  - 63
    EP  - 67
    PB  - Science Publishing Group
    SN  - 2578-8930
    UR  - https://doi.org/10.11648/j.cnn.20210503.16
    AB  - Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare neurodegenerative disorder characterized by rapidly progressive dementia and myoclonus. Additional clinical features include visual disturbances, cerebellar, and pyramidal/extrapyramidal signs. The rarity of this disease and the wide variety of initial symptoms make the early diagnosis quite challenging. Nevertheless, it can also be diagnosed in a centre with limited resources through good clinical analysis. Here a case of a 53-year-old man is presented who had a 6 weeks history of cognitive deterioration and myoclonus. Brain magnetic resonance imaging (MRI) showed ribbon-like areas of hyperintensity in the bilateral cortex on diffusion-weighted imaging (DWI); bilateral hyperintense caudate nucleus and putamen on T2-weighted and Fluid-attenuated inversion recovery (FLAIR) images. Electroencephalogram (EEG) showed intermittent generalized periodic sharp waves of triphasic morphology. The diagnosis of probable sCJD was reached based on the clinical features and characteristic findings in his MRI and EEG according to WHO criteria. Several other works of the literature were also reviewed for early diagnosis of sCJD and for the exclusion of other differential diagnoses which may mimic sCJD.
    VL  - 5
    IS  - 3
    ER  - 

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Author Information
  • Neurology, National Institute of Neurosciences and Hospital, Dhaka, Bangladesh

  • Neurology, National Institute of Neurosciences and Hospital, Dhaka, Bangladesh

  • Neurology, National Institute of Neurosciences and Hospital, Dhaka, Bangladesh

  • Neurology, National Institute of Neurosciences and Hospital, Dhaka, Bangladesh

  • Endocrinology, National Institute of Neurosciences and Hospital, Dhaka, Bangladesh

  • Neurology, National Institute of Neurosciences and Hospital, Dhaka, Bangladesh

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