Characterization of Creutzfeldt-Jakob Disease at the National Institute of Neurology and Neurosurgery. Havana, Cuba

Authors

Keywords:

NEURODEGENERATIVE DISEASES, PRIONS, CREUTZFELDT-JAKOB SYNDROME, ENCEPHALOPATHY, SPONGIFORM.

Abstract

Introduction: Creutzfeldt-Jakob disease (CJD) is a neurodegenerative disorder caused by prions in humans. It presents as a spongiform encephalopathy of worldwide distribution with a prevalence of 1 in 1,000,000 inhabitants.

Objective: to characterize Creutzfeldt-Jakob disease at the National Institute of Neurology and Neurosurgery during the period from February 1981 to January 2019.

Methods: an observational, descriptive cross-sectional study was carried out on patients diagnosed with Creutzfeldt-Jakob disease, of non-hereditary etiology, at the National Institute of Neurology and Neurosurgery of Cuba between 1981 and 2019. The universe was constituted by the 12 patients diagnosed in that period.

Results: female sex predominated in 66,3 %. The mean hospital stay was 9,8 days with a minimum of 7 and a maximum of 46 days. The first symptom identified by the patient was difficulty walking (50 %), followed by loss of balance (33 %) and dizziness (17 %). The predominant radiological finding was frontoparietotemporal and cerebellar atrophy in 50 % of patients. The most frequent clinical manifestations were aphasia in 25 %, disorientation, dysarthria, dysphagia and urination disorders in the same proportion.

Conclusions: In the investigation a greater number of cases were observed in the female sex. Difficulty walking followed by loss of balance and dizziness were the first symptoms identified. Frontoparietotemporal and cerebellar atrophy predominated as radiological findings. The most frequent clinical manifestations were aphasia, disorientation, dysarthria, dysphagia and urination disorders.

 

Downloads

Download data is not yet available.

Author Biography

Emmanuel Zayas Fundora, Universidad de Ciencias Médicas de la Habana. Facultad de Ciencias Médicas ‘’Manuel Fajardo’’. La Habana, Cuba.

Estudiante de 5to año de Medicina. Alumno Ayudante de Neurologia.

References

1. Exeni Díaz G., Costa M. , Salman J., Ávila S. Creutzfeldt-Jakob Syndrome of Genetic Origin: Series of Cases in the Argentinian Patagonia. Journal of Basic Applied Genetics. [Internet] 2020 [citado 12/10/21]; XXXI(1): 7-13. Disponible en: https://sag.org.ar/jbag/wp-content/upload/2020/05/BAG_VXXXl_1_2020_ART1_WEB.pdf

2. Uttley L, Carroll C, Wong R, Hilton DA, Stevenson M. Creutzfeldt-Jakob disease: a systematic review of global incidence, prevalence, infectivity, and incubation. Lancet Infect Dis. [Internet] 2020 [citado 12/10/21]; 20(1): e2-10. Disponible en: https://pubmed.ncbi.nlm.nih.gov/31876504/

3. Jones E, Mead S. Genetic risk factors for Creutzfeldt-Jakob disease. Neurobiology of Disease. [Internet] 2020 [citado 12/10/21]; 142: 104973. Disponible en: https://doi.org/10.1016/j.nbd.2020.104973

4 Munné Díaz C, Grau Solà M, Torreño Sánchez M. Enfermedad de Creutzfeldt-Jacob. Rev Cient Soc Esp Enferm Neurol. [Internet] 2013 [citado 12/10/21]; 37(1): 29-32. Disponible en: https://www.elsevier.es/es-revista-revista-cientifica-sociedad-espanola-enfermeria-319-articulo-enfermedad-creutzfeldt-jacob-S2013524613000093

5 Wang H, Rhoads DD, Appleby BS. Human prion diseases. Curr Opin Infect Dis. [Internet] 2019 [citado 12/10/21]; 32(3): 272-6. Disponible en: https://pubmed.ncbi.nlm.nih.gov/31008724/

6. Yang J, Kuang H, Wang Q, Liu J, Chen X, Shang H. Analysis of Chinese patients with sporadic Creutzfeldt-Jakob disease. Prion [Internet]. 2020 [citado 12/10/21]; 14(1): 137-42. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7219432/

7. Qi C, Zhang J-T, Zhao W, Xing X-W, Yu S-Y. Sporadic Creutzfeldt-Jakob Disease: A Retrospective Analysis of 104 Cases. Eur Neurol [Internet]. 2020 [citado 12/10/21]; 83(1): 65-72. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7265760/

8. Kotkowski E, Cabot JH, Lacci JV, Payne DH, Cavazos JE, Romero RS, et al. Creutzfeldt-Jakob Disease: In-hospital demographics report of national data in the United States from 2016 and review of a rapidly-progressive case. Clin Neurol Neurosurg [Internet]. 2020 [citado 12/10/21]; 197: 106103. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7703375/

9. Ustáriz García CR, Morera Barrios LM, Hernández Ramírez P, Estrada del Cueto M, Bencomo Hernández A, García García M de los A, et al. Origen y composición genética de la población cubana. Rev Cubana Hematol Inmunol Hemoter [Internet]. 2011 [citado 12/10/21]; 27(3): 273-82. Disponible en: http://scielo.sld.cu/scielo.php?script=sci_arttext&pid=S0864-02892011000300002

10. Hermann P, Treig J, Unkel S, Goebel S, Bunck T, Jünemann M, et al. Sporadic Creutzfeldt-Jakob Disease among Physicians, Germany, 1993-2018. Emerg Infect Dis. [Internet] 2020 [citado 12/10/21]; 26(8). Disponible en: https://wwwnc.cdc.gov/eid/article/26/8/19-1159_article

11. Brandel JP, Vlaicu MB, Culeux A, Belondrade M, Bougard D, Grznarova K, et al. Variant Creutzfeldt–Jakob Disease Diagnosed 7.5 Years after Occupational Exposure. N Engl J Med [Internet]. 2020 [citado 12/10/21]; 381(1): 1-3. Disponible en: https://pubmed.ncbi.nlm.nih.gov/32609989/

12. Martheswaran T, Desautels JD, Moshirfar M, Shmunes KM, Ronquillo YC, Hoopes PC. A Contemporary Risk Analysis of Iatrogenic Transmission of Creutzfeldt-Jakob Disease (CJD) via Corneal Transplantation in the United States. Ophthalmol Ther [Internet]. 2020 [citado 12/10/21]; 9(3): 465-83. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7406626/

13. Qavi AH, Imran TF, Hasan Z, Ilyas F, Ghani U, Assad S, et al. Serial Magnetic Resonance Imaging in Creutzfeldt-Jakob Disease: a Case Report and Literature Review. Cureus. [Internet] 2017 [citado 12/10/21]; 9(3): e1095. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5392034/

14. Giró MLH, González MA, Herrera AD, Rojas NBA, García RDLN, Almeida JC. Prevalencia de la Hipertensión Arterial en trabajadores de una institución de salud. Revista Cubana de Cardiología y Cirugía Cardiovascular [Internet]. 2017 [citado 12/10/21]; 23(2): 322-7. Disponible en: http://www.revcardiologia.sld.cu/index.php/revcardiologia/article/view/697/html_82

15. Sacco S, Paoletti M, Staffaroni AM, Kang H, Rojas J, Marx G, et al. Multimodal MRI staging for tracking progression and clinical-imaging correlation in sporadic Creutzfeldt-Jakob disease. Neuroimage Clin [Internet]. 2021 [citado 12/10/21]; 30: 102523. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7906895/

16. Sitammagari KK, Masood W. Creutzfeldt Jakob Disease. Treasure Island (FL): StatPearls [Internet]. 2022 [citado 12/10/21]. Disponible en: http://www.ncbi.nlm.nih.gov/books/NBK507860/

17. Younes K, Rojas JC, Wolf A, Sheng‐Yang GM, Paoletti M, Toller G, et al. Selective vulnerability to atrophy in sporadic Creutzfeldt‐Jakob disease. Ann Clin Transl Neurol [Internet]. 2021 [citado 12/10/21]; 8(6): 1183-99. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8164858/

18. Rhoads DD, Wrona A, Foutz A, Blevins J, Glisic K, Person M, et al. Diagnosis of prion diseases by RT-QuIC results in improved surveillance. Neurology [Internet]. 2020 [citado 12/10/21]; 95(8): e1017-26. Disponible en: https://pubmed.ncbi.nlm.nih.gov/32571851/

19. Mahboob HB, Kaokaf KH, Gonda JM. Creutzfeldt-Jakob Disease Presenting as Expressive Aphasia and Nonconvulsive Status Epilepticus. Case Rep Crit Care [Internet]. 2018 [citado 12/10/21]; 2018: 5053175. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5832162/

Published

2022-11-03

How to Cite

1.
Zayas Fundora E, Salgado Fuentes CE, Afonso Domínguez MK, Machado Cueto MF, Yllodo Cristo A, Leyva Pupo LV. Characterization of Creutzfeldt-Jakob Disease at the National Institute of Neurology and Neurosurgery. Havana, Cuba. Rev Ciencias Médicas [Internet]. 2022 Nov. 3 [cited 2025 Aug. 16];26(5):e5619. Available from: https://revcmpinar.sld.cu/index.php/publicaciones/article/view/5619

Issue

Section

ORIGINAL ARTICLES