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Neurology
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Sporadic Creutzfeldt- Jakob Disease: Case Series of Refractory Convulsive Status Epilepticus and Ataxic Variant in a Health Care Provider

  • Clinica Medical Neuro Center, Santa Cruz de la Sierra, Bolivia

Corresponding author: Enzo M. Fortuny — correspondence via editorial@ijcinnovation.org

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Volume 1, Issue 1

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  1. Received
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Abstract

Background: Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare, rapidly progressive, and fatal prion disease characterized by cognitive decline, myoclonus, cerebellar dysfunction, and characteristic neuroimaging abnormalities. Seizures are uncommon, and refractory convulsive status epilepticus represents a particularly rare complication. Case Presentations: The first case involved a 35-year-old man with several months of behavioral changes, sleep disturbances, gait instability, ataxia, dysarthria, and generalized myoclonus. Brain MRI demonstrated bilateral caudate and basal ganglia diffusion abnormalities with cortical involvement, and CSF 14-3-3 testing provided supportive evidence for probable sCJD. On hospital day 3, he developed recurrent generalized tonic-clonic seizures progressing to refractory convulsive status epilepticus requiring intubation, continuous EEG monitoring, levetiracetam, phenobarbital, midazolam, and propofol for seizure control. The second case involved a 50-year-old man with progressive sleep and behavioral disturbances followed by dysarthria and profound ataxia. MRI demonstrated bilateral caudate and lenticular hyperintensities with pulvinar and medial thalamic involvement producing a hockey-stick pattern. CSF 14-3-3 was positive, supporting a clinical suspicion of sCJD, although RT-QuIC and neuropathological confirmation were unavailable. Conclusions: These cases illustrate two clinically suspected presentations of sCJD, including one complicated by refractory convulsive status epilepticus and another with an ataxic-predominant phenotype. Acute deterioration, new seizure activity, or worsening myoclonus in patients with suspected sCJD should prompt EEG evaluation because status epilepticus, although rare, may require aggressive neurocritical care management.

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References

  1. 1. Ritchie DL, Smith C. Pathological spectrum of sporadic Creutzfeldt–Jakob disease. Pathology. 2025;57(2):196-206.
  2. 2. Bhatia P, Sonbol M, Jain D, Rincon-Flores N, Frontera A. Status Epilepticus in Creutzfeldt-Jakob Disease: A Case Report of an Unusual Presentation. Cureus. 2022;14(6):e26470.
  3. 3. Holt C, Sadek AT, Woodard J, Grinstead R. An Atypical Presentation of Sporadic Creutzfeldt-Jakob Disease in the Setting of Chronic Alcohol Use Disorder. Case reports in neurological medicine. 2025;2025:1332654.
  4. 4. Fragoso DC, Gonçalves Filho ALdM, Pacheco FT, Barros BR, Aguiar Littig I, Nunes RH, et al. Imaging of Creutzfeldt-Jakob Disease: Imaging Patterns and Their Differential Diagnosis. RadioGraphics. 2017;37(1):234-57.
  5. 5. Muayqil T, Gronseth G, Camicioli R. Evidence-based guideline: Diagnostic accuracy of CSF 14-3-3 protein in sporadic Creutzfeldt-Jakob disease. Neurology. 2012;79(14):1499-506.
  6. 6. Katsikaki G, Dagklis IE, Angelopoulos P, Ntantos D, Prevezianou A, Bostantjopoulou S. Atypical and early symptoms of sporadic Creutzfeldt - Jakob disease: case series and review of the literature. Int J Neurosci. 2021;131(9):927-38.
  7. 7. Uttley L, Carroll C, Wong R, Hilton DA, Stevenson M. Creutzfeldt-Jakob disease: a systematic review of global incidence, prevalence, infectivity, and incubation. The Lancet Infectious Diseases. 2020;20(1):e2-e10.
  8. 8. Watson N, Hermann P, Ladogana A, Denouel A, Baiardi S, Colaizzo E, et al. Validation of Revised International Creutzfeldt-Jakob Disease Surveillance Network Diagnostic Criteria for Sporadic Creutzfeldt-Jakob Disease. JAMA Network Open. 2022;5(1):e2146319-e.
  9. 9. Shimamura MI, Satoh K. Challenges and Revisions in Diagnostic Criteria: Advancing Early Detection of Prion Diseases. Int J Mol Sci. 2025;26(5).
  10. 10. Kojima G, Tatsuno BK, Inaba M, Velligas S, Masaki K, Liow KK. Creutzfeldt-Jakob disease: a case report and differential diagnoses. Hawaii J Med Public Health. 2013;72(4):136-9.
  11. 11. Green AJE. RT-QuIC: a new test for sporadic CJD. Pract Neurol. 2019;19(1):49-55.
  12. 12. Neri S, Mastroianni G, Gardella E, Aguglia U, Rubboli G. Epilepsy in neurodegenerative diseases. Epileptic Disorders. 2022;24(2):249-73.
  13. 13. Katramados AM, Burdette D, Patel SC, Schultz LR, Gaddam S, Mitsias PD. Periictal diffusion abnormalities of the thalamus in partial status epilepticus. Epilepsia. 2009;50(2):265-75.
  14. 14. Hufnagel A, Weber J, Marks S, Ludwig T, De Greiff A, Leonhardt G, et al. Brain diffusion after single seizures. Epilepsia. 2003;44(1):54-63.
  15. 15. Jurcau MC, Jurcau A, Diaconu RG, Hogea VO, Nunkoo VS. A Systematic Review of Sporadic Creutzfeldt-Jakob Disease: Pathogenesis, Diagnosis, and Therapeutic Attempts. Neurol Int. 2024;16(5):1039-65.

Supplementary material

Declarations

Funding

This study was not supported by any sponsor or funder.

Conflicts of interest

The authors declare no conflicts of interest

Author contributions

Enzo M. Fortuny; Writing original and revised drafts. Andres Jordan Camargo; Data Collection and clinical management. Erick Gonzalez; Data Collection and patient management. Laura Alejandra Ochoa Torrico; Analysis, supervision, data collection, writing – review and editing. Fatima Urquieta Zabala; Analysis, supervision, data collection. Mario Camargo; Conceptualization.

Data availability

The data that support the findings of this study are not publicly available due to patient and family privacy concerns but are available from the corresponding author upon reasonable request.

Ethics approval

Not Required / Not Applicable

Consent for publication

Obtained

How to Cite

Enzo M. Fortuny, Laura Alejandra Ochoa Torrico, Mario Camargo Villarreal, Erick Gonzalez Delgado, Fatima Urquieta Zabala, Andres Camargo Jordan. Sporadic Creutzfeldt- Jakob Disease: Case Series of Refractory Convulsive Status Epilepticus and Ataxic Variant in a Health Care Provider. Int J Clin Innov. 2026;1(1).

Received July 15, 2026 · Accepted September 7, 2026

© The Author(s). This is an open-access article distributed under the terms of the Creative Commons Attribution 4.0 International (CC BY 4.0).