Abstract
BackgroundCreutzfeldt-Jakob disease is a rare and fatal neurodegenerative disorder that affects mammals and humans. The prevalence of this disease in the United States is 0.5 to 1 per million inhabitants. So far in Ecuador, we do not know what the prevalence or incidence is, and only one case report has been written.Case presentationWe present a case series of Creutzfeldt-Jakob disease in a third-level hospital in Quito. The average age of symptom onset in our patients was 58.8 years. The male to female ratio was 1:1. Two patients began with cognitive/behavioral symptoms, while 4 patients began with focal neurological signs; 1 case with ataxia, 2 with gait disorders and 1 with vertigo and headache. All of the patients had the clinical features established by the World Health Organization. In addition, the entire cohort was positive for the 14–3-3 protein in cerebrospinal fluid, and had high signal abnormalities in caudate and putamen nucleus in DWI and FLAIR IRM. Only in one case, did we reach a definitive diagnosis through a pathological study. All other cases had a probable diagnosis. In this series of cases, 6 out of 6 patients died. The average time from the onset of the symptoms to death in this cohort was 13 months.ConclusionThis is the first report of a series of cases of Creutzfeldt-Jakob disease in Quito. Although definitive diagnosis must be histopathological, there are ancillary tests currently available that have allowed us to obtain a diagnosis of the disease.
Highlights
Creutzfeldt-Jakob disease is a rare and fatal neurodegenerative disorder that affects mammals and humans
Definitive diagnosis must be histopathological, there are ancillary tests currently available that have allowed us to obtain a diagnosis of the disease
We present 6 case reports of Creutzfeldt-Jakob disease (CJD) diagnosed between January 2012 and June 2016 at Carlos Andrade Marín Hospital (CAMH)
Summary
These are the first case reports of CJD in Ecuador from a third level hospital of Quito. All other cases had a probable diagnosis This disease should be considered in individuals older than 50 years of age, with a rapidly progressive dementia associated with myoclonus, visual symptoms, and ataxia accompanied by signs of pyramidal and extrapyramidal dysfunction. BM participated in designing the project and wrote the manuscript. Ethics approval and consent to participate The study protocol was approved by the Carlos Andrade Marín Hospital ethics committee. Written informed consent of participation was obtained from the legal guardians. Consent for publication Written informed consent to publish this case reports was given by the legal guardians. Author details 1Hospital Carlos Andrade Marín, Av. 18 de Septiembre y Ayacucho, Quito, Ecuador. Author details 1Hospital Carlos Andrade Marín, Av. 18 de Septiembre y Ayacucho, Quito, Ecuador. 2Universidad Central del Ecuador, Calle Iquique y Sodiro, Quito, Ecuador. 3Facultad de Medicina de la Pontifica Universidad Católica del Ecuador, Avenida 12 de Octubre y Vicente Ramón Roca, Quito, Ecuador
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
