Enfermedad de Creutzfeldt-Jakob: experiencia de 5 años en un hospital terciario de Chile
- Autores: Sergio Legua, Paula Castillo-Torres, Alfonso León-Mantero, Manuel Alvarado-Pastenes, Gladys Godoy-Reyes, David Sáez-Méndez, José Luis Bucarey-Tapia
- Localización: Revista Médica de Chile, ISSN-e 0034-9887, Vol. 149, Nº. 9, 2021, págs. 1285-1291
- Idioma: español
- Títulos paralelos:
- Creutzfeldt-Jakob disease, experience in 17 patients
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Resumen
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.
