Izaro Kortazar-Zubizarreta, Rebeca Ruiz-Onandi, Arrate Pereda, et al. - Along with rapid progression and great variability, patients with sporadic Creutzfedlt-Jakob disease have heterogeneous clinical presentation and median survival times from diagnosis to death of 4 to 6 months. Researchers herein report a case of sporadic Creutzfeldt-Jakob disease with the longest survival period ever documented (survival period of 14 years). The case was of a 61-year-old woman with a history of initially rapidly progressive dementia, with subsequent development of pyramidal and extrapyramidal signs. No significant alterations were identified in the initial magnetic resonance imaging evaluation, single-photon emission computed tomography and electroencephalogram. In the postmortem examination of the brain, researchers identified diffuse spongiform change, gliosis, and neuronal loss along with abnormal immunostaining of prion protein in the grey matter, especially in the cerebellum. The unusually long duration of survival in this case highlights the relevance of histopathological confirmation with brain autopsies for suspected cases, as the disease can be easily misdiagnosed when dealing with such slowly progressing cases.