| Revista: | Revista médica de Chile |
| Base de datos: | PERIÓDICA |
| Número de sistema: | 000449348 |
| ISSN: | 0034-9887 |
| Autores: | Legua Koc, Sergio1 Castillo Torres, Paula2 León Mantero, Alfonso3 Alvarado Pastenes, Manuel3 Godoy Reyes, Gladys3 Sáez Méndez, David3 Bucarey Tapia, José Luis4 |
| Instituciones: | 1Servicio de Salud Aconcagua, Hospital San Camilo, San Felipe, San Felipe de Aconcagua. Chile 2Servicio Metropolitano Occidente, Hospital San Juan de Dios, Santiago de Chile. Chile 3Universidad de Chile, Facultad de Medicina, Santiago de Chile. Chile 4Universidad de Valparaíso, Facultad de Medicina, San Felipe, Valparaíso. Chile |
| Año: | 2021 |
| Volumen: | 149 |
| Número: | 9 |
| Paginación: | 1285-1291 |
| País: | Chile |
| Idioma: | Español |
| Tipo de documento: | Artículo |
| Enfoque: | Analítico, descriptivo |
| Resumen en inglés | Background: Creutzfeldt-Jakob disease (CJD) is a prion affection that typically produces a rapidly progressive dementia with different neurologic and extra-neurologic manifestations. Aim: To characterize clinical, imaging and electroencephalography findings in patients with a probable CJD. Patients and Methods: A case series study of patients admitted in the Neurology department at a public hospital, between 2014 and 2019. Demographic, clinical, imaging, and electroencephalographic data of patients with probable CJD were analyzed. Results: Seventeen patients aged 63 ± 11 years (53% women) with a probable CJD were gathered. The incidence was 4.7 cases/year per million inhabitants. Twenty four percent of patients had a family history of CJD. The median time between the onset of symptoms and the hospital admission was three months with a survival of four months. The most common clinical manifestations were an amnesic syndrome in 88%, myoclonus in 76%, frontal syndrome and ataxia in 71%. Brain MRI was abnormal in all patients. The preponderant finding was the involvement of the caudate nucleus in 82% of cases. In the EEG, 94% of patients had abnormalities. All had a theta-delta slowing as a base rhythm. The pseudo-periodic pattern was observed in the 29% and status epilepticus in 18%. Conclusions: In this group of patients we observed the heterogeneity of the clinical manifestations of the disease, the frequent imaging and electroencephalographic alterations and the short evolution time leading to death |
| Disciplinas: | Medicina |
| Palabras clave: | Neurología, Priones, Enfermedad de Creutzfeldt-Jakob, Demencia, Status epilepticus |
| Keyword: | Neurology, Prions, Dementia, Creutzfeldt-Jakob disease, Status epilepticus |
| Texto completo: | Texto completo (Ver HTML) Texto completo (Ver PDF) |
