Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2024 Mar 30;55(1):e2035821.
doi: 10.25100/cm.v55i1.5821. eCollection 2024 Jan-Mar.

Sporadic Creutzfeldt Jakob disease: Case series in Peru

Stefany Espinoza  1 , Diego Canales  1 , Cecilia Calderón  2 , David Diaz  1 , Elliot Barreto-Acevedo  1
Affiliations
Case Reports

Sporadic Creutzfeldt Jakob disease: Case series in Peru

Stefany Espinoza et al. Colomb Med (Cali). .

Abstract
in English, Spanish

Description of the cases: A series of 6 cases with a probable diagnosis of sporadic CJD, treated in a Peruvian national reference hospital, are presented.

Clinical findings: The relevant clinical signs were rapidly progressive dementia and myoclonus, followed by akinetic mutism and pyramidal signs.

Treatment and results: Of the cases presented, 80% were men, with an average age of presentation of 65 years and duration from diagnosis to death of 6.5 months. Laboratory tests, images (Brain Resonance) and protein dosage 14.3.3 were performed to support the clinical suspicion. There is no effective treatment at the moment for said pathology.

Clinical relevance: Creutzfeldt-Jakob disease (CJD) is a progressive, fatal, neurodegenerative disease of low prevalence and incidence. Great clinical suspicion and the exclusion of other etiologies are required. Currently there is no treatment for this entity and there is a high probability of death before one year.

Descripcion de los casos: Se presenta una serie de 6 casos con diagnóstico probable ECJ esporádica, atendidos en un hospital peruano de referencia nacional.

Hallazgos clínicos: Los signos clinicos relevantes fueron la demencia rapidamente progresiva y las mioclonias , seguidas del mutismo acineticos y signos piramidales.

Tratamiento y resultados: De los casos presentados , el 80% fueron varones , con edad de presentación promedio a los 65 años y tiempo de duración desde el diagnostico hasta el deceso de 6.5 meses . Se realizo examenes de laboratorio, imágenes ( Resonancia Cerebral) y dosaje de proteina 14.3.3 para apoyo a la sospecha clinica .No se cuenta con un tratamiento efectivo al momento para dicha.

Relevancia clinica: La enfermedad de Creutzfeldt-Jakob (ECJ) es una enfermedad neurodegenerativa, progresiva, mortal, de baja prevalencia e incidencia . Se requiere de gran sospecha clínica y la exclusión de otras etiologías. Actualmente no hay un tratamiento para esta entidad y hay alta probabilidad de muerte antes del año.

Keywords: Creutzfeldt-Jakob Syndrome; Dementia; Peru; Prion Diseases; Prions.

PubMed Disclaimer

Conflict of interest statement

Conflict of interest: None

Figures

Figure 1
Figure 1. A) Brain MRI FLAIR and diffusion: Hypersignal and restriction, respectively, in caudate and putamen (Case 1). B) Brain MRI: Slight increase of intensity in FLAIR at the level of bilateral caudate nuclei, showing diffusion restriction. Moderate cerebral atrophy (Case 4).
Figure 2
Figure 2. EEG of cases 2 (A) and 3 (B). On slowed (A and B) and attenuated (B) background, periodic generalized discharges to 0.5-1 Hz (A) y 0.8-1.2 Hz (B).
Figura 1
Figura 1. A) RM de encéfalo FLAIR y difusión: Hiperseñal y restricción, respectivamente, en caudados y putámenes (Caso 1). B) RM de encéfalo: Leve incremento de intensidad en FLAIR a nivel de núcleos caudados bilaterales, mostrando restricción a la difusión. Moderada atrofia cerebral (Caso 4)
Figura 2
Figura 2. EEG de casos 2 (A) y 3(B). Sobre fondo lentificado (Ay B) y atenuado (B),descargas generalizadas periódicas a 0.5-1 Hz (A) y 0.8-1.2 Hz (B).
See this image and copyright information in PMC

References

    1. Baldwin KJ, Correll CM. Prion Disease. Seminars Neurol. 2019;39(4):428–439. - PubMed
    1. Fragoso DC. Gonçalves Filho AL da M.Pacheco FT Imaging of Creutzfeldt-Jakob Disease imaging patterns and their differential diagnosis. RadioGraphics. 2017;37(1):234–257. - PubMed
    1. Tee BL, Longoria Ibarrola EM, Geschwind MD. Prion Diseases. Neurol Clin. 2018;36(4):865–897. - PubMed
    1. Wang H, Rhoads D, Appleby B. Human prion diseases. Current Opinion Infect Dis. 2019;32(3):272–276. - PubMed
    1. Uttley L, Carroll C, Wong R, Hilton DA, Stevenson M. Creutzfeldt-Jakob disease a systematic review of global incidence, prevalence, infectivity, and incubation. Lancet Infectious Diseases. 2020;20(1):e2–10. - PubMed

Publication types

Supplementary concepts

LinkOut - more resources