To assess time of survival in patients diagnosed with Creutzfeldt-Jakob disease (CJD) that were admitted to a referral CJD disease surveillance center in Argentina and evaluate the impact of clinical manifestations, ancillary study findings and treatment on it. 

We reviewed 200 medical records of patients with possible and probable CJD diagnosis from the Brain bank dataset. We included subjects with probable and definitive diagnosis of CJD based on WHO criteria, registered between July 2011 and July 2021. We analyzed data about demographic variables, symptoms, MRI, EEG, protein 14.3.3, t-tau and p-tau in CSF, treatment and survival.

We included 64 patients, age 64± 9. The delay to diagnosis from the first symptom was 13.1 weeks (w) CI95% [11.7,19.1] with the cognitive variant presenting a significantly longer delay 24.3w CI95% [12.14,52.4] p=0.021.  The median survival time was 66w CI 95%[44.0,150.3] . 23 patients (35%) received intravenous methylprednisolone treatment and 4 patients received intravenous immunoglobulin. Neither modified survival time (p=0.9). The administration of antipsychotics was associated with survival decrement (HR 3.1 CI 95%[1.3-7.3], p=0.011).

These results show the devastating course of the disease, with half of the patients not surviving 16 months after the first symptom. More importantly, 3 months go by without diagnosis. It is necessary to optimize the diagnostic accuracy because the implementation of certain therapeutic measures (antipsychotics) could worsen survival.