• Sergio Legua-Koc, Paula Castillo-Torres, Alfonso León-Mantero, Manuel Alvarado-Pastenes, Gladys Godoy-Reyes, David Sáez-Méndez, and José Luis Bucarey-Tapia.
• Servicio de Salud Aconcagua, Servicio de Neurología Hospital San Camilo, San Felipe, Chile.
• Rev Med Chil. 2021 Sep 1; 149 (9): 1285-1291.

BackgroundCreutzfeldt-Jakob disease (CJD) is a prion affection that typically produces a rapidly progressive dementia with different neurologic and extra-neurologic manifestations.AimTo characterize clinical, imaging and electroencephalography findings in patients with a probable CJD.Patients And MethodsA 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.ResultsSeventeen 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%.ConclusionsIn 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.

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