Experiencia Con La Enfermedad De Creutzfeldt-Jakob De Un Único Centro De Referencia En México. Análisis De Una Serie De Casos

Introduction: Creutzfeldt-Jakob disease (CJD) is a rapidly progressive and fatal central nervous system disease caused by prions.Objective: To present the main clinical and paraclinical characteristics of patients with probable CJD in a referral center of Latin America.Methods: Retrospective study of patients diagnosed with rapidly progressive dementia between 2014 and 2019.Clinical, demographic, electroencephalogram, magnetic resonance imaging, and 14-3-3 protein characteristics were included, as well as positron-emission tomography (PET) data when available.Results: Twenty-four patients met the criteria for sporadic CJD (75% were women).Mean age was 59.29 ± 11.67 years, while mean disease duration from symptom onset to hospital admission was 7.41 ± 6.54 months.The most common first symptom was behavioral changes (41.7%).Delta wave complexes prevailed (54.2%) on electroencephalogram, cortical hyperintensity (83.3%) on magnetic resonance and frontal hypometabolism (37.5%) on PET.Seven cases showed positive total Tau; five, positive 14-3-3 protein; and three, positive phosphorylated tau on cerebrospinal fluid analysis.Conclusions: There is significant clinical heterogeneity regarding initial symptoms.Auxiliary test findings were consistent with those of other series.